1. CONGENITAL SYPHILIS SINDHU E. PHILIP, MD. DEPARTMENT OF PEDIATRICS
09/20/02

2. Introduction
Infant usually infected in utero by transplacental passage of
Treponema pallidum from infected mother at any time.
Infection may also occur from contact with an infectious
lesion during passage through the birth canal
It remains unclear what factors determine which mothers,
particularly those in the latent stage, will pass the disease to
the fetuses.
Also unclear why some infants, infected in utero, are born
asymptomatic, but develop overt dz. In first few wks./mo.

3. Epidemiology Worldwide, but more frequent in urban areas.
Incidence of congenital syphilis has dramatically decreased
in the U.S. since 1991.

4. Infection can be transmitted to fetus at any stage of disease.
Rate of infection 60% - 100% during second stage.
Transmission rates slowly decreases with increasing duration of the disease.
Women, untreated early syphilis: 40%
of pregnancies result in spontaneous abortion, stillbirth, or perinatal death.

5. Pathogenesis Infection before 4th month of pregnancy?
Possible that treponemes do in fact pass from mother
to fetus before 5th month of gestation, but classic
pathologic changes do not occur until after 5th month.
Infection involves the placenta, and spreads
hematogenously to the fetus, widespread involvement
is characteristic. Infected placenta is paler, thicker,
and larger than normal.

6. Clinical Manifestations
Damage to fetus depends on the stage of development
at which infection has taken place and time elapsed
before treatment.
Early infection, untreated: miscarriage, stillbirth, neonatal
death, IUGR, premature delivery.
Survivors:
Early congenital syphilis: clinical manifestations within first
2 years of life
Late congenital syphilis: clinical manifestations after 2yo.

7. Early Congenital Syphilis
Early manifestations are varied, with multi-system
Involvement
Hepatosplenomegaly- diffuse inflammation, scarring
Jaundice – due to hepatitis
Generalized lymphadenopathy –epitrochclear nodes
Coombs – hemolytic anemia, thromobocytopenia, leukopenia, leukocytosis
Hydrops fetalis
Mucocutaneous: rhinitis (highly infectious), “snuffles”, mucous patches
Macuolpapular rash
Desquamation
Pemphigus syphiliticus (vesicular bullous eruptions of palms and soles)
Petechial lesions
Bony lesions, osteochondritis, periostitis, pseudoparalysis
Syphilitc leptomeningitis
Chorioretinitis,salt and pepper fundus, glaucoma
Pancreatitis

8. Papulosquamous Plaques

9. Broken vesicles, desquamation
Condylomata around anus
Infiltration, desquamation and rhinitis

10. Rhinitis (snuffles), mucous patches, damage to palate(late)
Bullae and vesicular rash on soles
Eroded papular lesions

11. Lone Bone Radiographs Osteochondritis of distal radius and ulna
Osteochondritis of femur and tibia
metaphysis

12. Late Congenital Syphilis
Results primarily from chronic inflammation of bone, teeth,
and CNS.
Interstitial keratitis (inflammatory)
Nerve deafness
Clutton’s Joints (synositis, restricted movement)
Hutchinson’s triad (teeth, intersitial keratitis, 8th nerve deafness)
Mulberry molars
Flaring scapulas
Hydrocephalus
Mental retardation
Frontal bossing
Saddle nose
Protruding mandible
High arched palate
Perioral fissures
Higouemenaki’s sign(periosteal rxn of clavicle, sternocleidomastoid)
Saber shins
Rhagades (linear scars that become fissured or ulcerated)

13. Hutchinson’s teeth – peg shaped upper incisors
Frontal Bossing

14. Saber shins:
Anterior bowing of tibias
Gumma:
Thin atrophic scar

15. Diagnosis Definitive diagnosis can be made by dark-field microscopy
on specimen from skin lesions, placenta, umbilicus.
Serologic tests are the principal means for diagnosis:
Nontreponemal test: VDRL, RPR
Treponemal tests: TPI, FTA-ABS, MHA-TP

16. Diagnosis Nontreponemal: VDRL, RPR
Quantitative results correlate with disease activity,
therefore helpful in screening.
Titers rise when disease is active, fall when
treatment is adequate
In congenital infxn. These tests become non- reactive
within a few months of adequate treatment.

17. Non-treponemal testing
Detects antibodies against a cardiolipin- cholestrol-
lecithin complex, not specific for syphilis.
False Positives:
Only in serum, not in CSF testing
certain viral infxns: infectious mononucleosis,
hepatitis, varicella, measles
lymphoma, TB, malaria, endocarditis,
connective tissue disease
pregnancy (?)
abuse of injection drugs

18. Diagnosis A sustained 4 fold decrease in titer of nontreponemal
test after treatment demonstrates adequate therapy.
A 4 fold increase titer increase after treatment suggests
reinfection or relapse.

19. Confirmatory Testing Treponemal Tests: TPI, FTA-Abs, MHA-TP
Treponemal antibody titers become positive soon
After initial infection and usually remain positive
For life, even with adequate therapy.
Antibody titers do not correlate with disease activity, and
are not quantified.
Not 100% specific for syphilis: other spirochetal dz,

20. Infant Testing
Reactive serology in neonate could be due to IgG passively
transferred to newborn through placenta, and does not indicate
active infection.
If infant’s titer higher than mother’s  congenital infection
If decreasing titer in infant  passive transfer of antibodies,
should disappear by 3-4 months of age.
Persistently reactive VDRL, with rising titer  Active Infection

21. Recommended Interpretation
Non-treponemal Test (VDRL, RPR, EIA, ART)
Treponemal Test (MHA-TP, FTA-ABS)
Mother
Infant
Interpretation -
No syphilis or incubating syphilis in the mother and infant. +
No syphilis in mother (false-positive non-treponemal test with passive transfer to infant).
+/-
Maternal syphilis with possible infant infection; or mother treated for syphilis during pregnancy; or mother with latent syphilis and possible infection of infant.
Recent or previous syphilis in the mother; possible infection in the infant.
Mother successfully treated for syphilis before or early in pregnancy; or mother with Lyme disease, yaws, or pinta (ie, false-positive serology).

22. CSF Examination CSF abnormalities may occur in congenital syphilis
Even in absence of neurologic involvement.
* Leukocytosis
* Elevated protein in CSF
* positive VDRL (no false positives)

23. Evaluation Csf Analysis for VDRL, cell count & protein Thorough P.E.
Maternal hx, results of serologic testing
Thorough P.E.
Long bone xrays
Non-treponemal AB titer
Treponemal AB titer
Head US
Csf Analysis for VDRL, cell count & protein
CBC with platlets
LFT’s UA
Chest xray
HIV antibody test
Opthalmologic exam

24. Treatment Proven or highly probable: Aqueous crystalline Penicillin G
100, ,000U/kg/day
(given q8-q12hrs) IV for
10 days OR
Procaine Penicillin G
50,000 U/kg/day IM for
10days
If >1 day of therapy missed, entire course should be restarted!

25. Treatment Asymptomatic, Normal CSF exam, CBC, platelets, and
Radiologic exam:
1. No maternal tx  aqueous PCN G IV for days
2. Tx w/ Erythromycin  clinical, serologic follow-up,
and Benzathine Pcn G IM x 1
3. Tx < 1month before
Delivery, or <4 fold
Decrease in titers  clinical, serologic follow-up and
Benzathine Pcn G IM x 1

26. Treatment Treat all newborns w/ positive VDRL as if they have
congenital syphilis, even if mother thought to not have an
active infection.
Difficult to document that mother received adequate tx,
and has falling VDRL titer.
2. Low titer VDRL test may be compatible with latent
maternal syphilis.
3. Newborn may not have clinical manifestations at birth.
4. Compliance with follow-up visits may be problem.

27. Follow-up Should have careful follow-up examination at
1, 2, 4, 6, and 12 months of age.
Serologic non-treponemal tests: 3, 6, 12 months, and end of tx
(or until non-reactive)
Non-treponemal Ab titers decline by 3 months of age, and
Should be Non-reactive by 6 months, if infant was not infected.
(transplacentally aquired antibodies.)
If persistent, stable titers, consider retreatment.
Congenital neurosyphilis- CSF exam at 6 month intervals until
normal

28. Research Advances
UT Southwestern Medical Center at Dallas research team has
develop 2 blood test that quickly and reliably diagnose
Congenital syphilis in newborns. (June, 2002)
Detect neonatal antibodies to the syphilis bacterium or the
DNA of the syphilis organism itself, as predictors of CNS
Invasion. (one day vs. ten days of PCN)
IgM Immunoblotting test PCR
In 1998 scientist at UTHSC in Houston, Institute for Genomic
Research in Maryland, mapped the 1.1 million base pairs of DNA that
make up the syphilis genome.Entire genomic pattern of Treponema
Pallidum now known. Difficult to study previously b/c of
dependence on mamalian host for viability. New breakthroughs?