1. Congenital Syphilis – in Theory and in Reality in Estonia, 1991-2005
Liis Toome
Tallinn Children’s Hospital

2. Incidence of Syphilis in 2001 per 100 000 Population
Estonia 2005 – 8,2/
EpiNorth Journal 2002, No 4

3. Congenital Syphilis in Estonia since 1991
Aim of the study:
to describe the epidemiology of congenital syphilis
in Estonia, (-2005)
Methods:
retrospective analysis of the cases of CS in children aged < 2 years
T. Rjabova, L. Toome
Tallinn Children’s Hospital
K. Kink
West Tallinn Central Hospital
E. Tamm
Children’s Clinic, Tartu University Hospitals
A. Kangur
North Estonia Regional Hospital

4. Health Protection Inspectorate, 2006
Syphilis in Estonia,
Number of cases .
Health Protection Inspectorate, 2006

5. The Incidence of Congenital Syphilis in Estonia, 1982 - 2005
years
< 1 year

6. Microbiology T. Pallidum A tightly coiled motile spirochete
Can be detected on dark-field microscopy
Has not been cultured in vitro
Treponema Pallidum
Treponema Pallidum on darkfield

7. Transmission Maternal infection (N = 428) Transmission rate Stillbirth
Live births with congenital syphilis
Untreated primary syphilis
29 %
3 %
26 %
Untreated secondary syphilis
59 %
20 %
39 %
Early latent disease
50 %
17 %
33 %
Late latent disease
13 %
5 %
8 %
Sheffield JS et al, Am J Obstet Gynecol, 1999

8. Early Congenital Syphilis - Clinical Manifestations
IUGR
Nonimmune hydrops fetalis
Enlarged placenta
Mucocutaneous manifestations
Persistent rhinitis (snuffles)
Maculopapular eruption
Superficial desquamation
Pemphigus syphiliticus
Condylomata lata

9. Early Congenital Syphilis - Clinical Manifestations
Jaundice, hepatosplenomegaly
syphilitic hepatitis
Generalized lymphadenopathy
Hematologic manifestations
hemolytic anemia, thrombocytopenia
Bone lesions
osteochondritis, -myelitis, periostitis
pseudoparalysis of Parrot
Pneumonitis, nephrotic syndrome
Syphilitic leptomeningitis
Ocular manifestations
chorioretinitis, glaucoma, cataract
Pneumonia alba
Metaphyseal dystrophy
Wimberger’s sign

10. Serologic Diagnosis in the Infant
“Nontreponemal Antibody Tests”
VDRL =
Veneral Disease Research Laboratory
RPR =
Rapid Plasma Reagin
Treponemal
Antibody Tests
TPHA =
T.pallidum hemagglutination test
FTA-ABS (IgM) =
Flourescent Treponemal Antibody IgM Test
IgM ELISA =
Enzyme-Linked Immunosorbent Assay
IgM Immunoblotting

11. Congenital Syphilis in Estonia, 1991-2005
Tallinn - 12 cases, Harjumaa - 7 cases, Lääne-Virumaa - 5 cases

12. Children’s Age at the Time of Diagnosis in Estonia, 1991-2005

13. Clinical Manifestations of Congenital Syphilis in Estonia, 1991-2005

14. Case 1 Newborn, syphilitic hepatitis
GA 36, BW 2529 g
jaundice from the birth
syphilitic hepatitis
hepatosplenomegaly
indirect bilirubin 245 mol/l
direct bilirubin 187 mol/l
elevated serum aminotransferases
Anemia, thrombocytopenia
Cardiolipin ag 4+
Treponemal ag 4+

15. Case 2 1 month 2 weeks, pseudoparalysis of Parrot
Bone lesions
with superimposed fractures
BW 2900 g
1 month
unexplained rhinitis
anemia Hgb 72 g/l, ER 2,5x1012
CRP 187 mg/l
pneumonia?
Jarisch-Herxheimer reaction
Pseudoparalysis of Parrot
Serology
RPR 1 : 40
TPHA 1 : 2560
Wimberger sign

16. Case 3 1 month 3 weeks, syphilitic glomerulonephritis
maculopapular rash, rhinitis, abdominal distension
“snuffles”, syphilitic ileitis
rectal bleeding
syphilitic glomerulonephritis with nephrotic syndrome
generalized edema + ascitis
5166 g  4154 g
macrohematuria
severe proteinuria
hepatosplenomegaly
panmetaphysitis
RPR 1 : 240, TPHA 1 : 640

17. Case 4 2 months, “asymptomatic”
BW 3250 g
Incarcerated inguinal hernia
Anemia  Hgb 77 g/l, ER 2,4 x 1012
Maculopapular eruption of the palms and soles, becoming coppery-brown
Hepato (+ 3,5 cm) spleno (+ 1,0 cm) megaly
Fever 38º
Jarisch-Herxheimer reaction, tº 40 º C
Serology
Cardiolipin ag 4+
Treponemal ag 4+

18. Case 5 6 months, Jarisch-Herxheimer Reaction
Term delivery, BW 2660 g
Persistent rhinitis
Mucocutaneous manifestations
Maculopapular eruption treated as allergic dermatitis and as scabies
Deep fissures radial to the angles of the mouth  rhagades
Hepatosplenomegaly, osteochondritis
SR 70 mm/h, Hgb 83 g/l
Jarisch-Herxheimer reaction
t° 39° C in 2 hours of treatment
RPR 1:640

19. Case 6 1 year 1 month, manifestations of CNS
BW 2731 g, parenteral abuse of alcohol
During the first year of life
Failure to thrive
Maculopapular rash - atopic dermatitis, scabies?
Anemia, hepatomegaly
1 year 1 month – 7,1 kg / 71 cm / OFC 44 cm
Mental retardation
Optic nerve atrophy
Brain CT – cortical atrophy
Serologic diagnosis
RPR 1 : 128
WB IgG positive
CSF FTA-Abs 2+

20. Treatment of the Newborn
Maternal Rx
Clinical Findings in Newborn
Drug
(Penicillin G)
Route
None or
inadequate
Present
Aqueous or
Procaine
IM/IV IM
10-14 days
Absent
Benzathine
Adequate (during pregnancy)
Benzathine (CDC)
Follow-up only (AAP)
Single dose
Adequate (before pregnancy)
Follow-up only Or
Remington & Klein, 2006

21. Post-treatment Follow-up
Patient Category
Follow-up Procedures
Infants
- diagnosed as having congenital syphilis
RPR testing every 2-3 mo until negative or decreased fourfold. If RPR titer is stable or increasing after 6-12 mo after treatment, reevaluate and re-treat.
Perform treponemal antibody test after age of 15 mo.
If CNS disease, repeat CSF evaluation every 6 mo until normal. With abnormal CSF on re-testing, re-treat.
Careful developmental evaluation, vision and hearing testing
who received treatment in utero or
at birth because of maternal syphilis
RPR testing at birth and then every 3 mo until result is negative.
Treponemal antibody test after age of 15 mo.
Recommendations for follow-up evaluation are summarirized in this table. IT is advisable to monitor the out-come of therapy by repeated RPR testing. Patients responding to therapy should have falling titres and as many as % should become seronegative within 1 year.
Rathbun KC, Sex Transm Dis 10:102, 1983

22. Late Congenital Syphilis - after the first two years of the life
Dentition
Hutchinson’s teeth
Eye
interstitial keratitis
Ear
eighth nerve deafness
Skin, face
rhagades, “saddle nose”
CNS
mental retardation, HC
Bones and joints
“saber shins”, “Clutton’s joints”
Hutchinson’s teeth
“Saber shins”
“Clutton’s joints”
Remington, 2006

23. Prevention Congenital syphilis is a preventable disease!
At least one serologic test for syphilis during the first trimester
For communities with high prevalence of syphilis
repeated testing at the beginning of the third trimester
at delivery (not in infants)
Adequate treatment of infants in utero or at birth
with subsequent follow-up

24. Conclusions from Estonian Experience
Political and social changes in the beginning of independent Estonian Republic brought about
the increase of incidence of syphilis
the cases of congenital syphilis
After 15 years congenital syphilis is a disappearing disease thanks to
the decreased incidence of syphilis in the population
The increased awareness of the importance of adequate prevention of transmission of the disease to the fetus and the newborn