Congenital Syphilis –in Theory
and in Reality in Estonia, 1991-2005
Liis ToomeTallinn Children’s Hospital
15.09.2006
Incidence of Syphilis in 2001 per 100 000 Population
EpiNorth Journal 2002, No 4
Estonia 2005 – 8,2/100 000
Congenital Syphilis in Estonia since 1991 Aim of the study:
to describe the epidemiology of congenital syphilis in Estonia, 1991-2001(-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
Syphilis in Estonia, 1971 - 2001
0
200
400
600
800
1000
1200
Health Protection Inspectorate, 2006
Number of cases
The Incidence of Congenital Syphilisin Estonia, 1982 - 2005
0
2
4
6
8
10
Health Protection Inspectorate Our cohort (n=28)
0 - 14 years < 1 year
Microbiology
T. Pallidum A tightly coiled motile spirochete Can be detected on dark-field
microscopy Has not been cultured in vitro
Treponema Pallidum on darkfield
Treponema Pallidum
TransmissionMaternal infection(N = 428)
Transmission rate
StillbirthLive 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
Early Congenital Syphilis -Clinical Manifestations
IUGR Nonimmune hydrops fetalis Enlarged placenta Mucocutaneous manifestations
Persistent rhinitis (snuffles) Maculopapular eruption Superficial desquamation Pemphigus syphiliticus Condylomata lata
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 dystrophyWimberger’s sign
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
Congenital Syphilis in Estonia, 1991-2005n = 28
Tallinn - 12 cases, Harjumaa - 7 cases, Lääne-Virumaa - 5 cases
Children’s Age at the Time of Diagnosisin Estonia, 1991-2005
1 1 14
21
1. month2. month3. month6. month2. year
(n = 28)
Clinical Manifestations of Congenital Syphilis in Estonia, 1991-2005
4 %
4 %
29 %
33 %
63%
39 %
32 %
0% 20% 40% 60% 80% 100%
CNS
renal manifestations
thrombocytopenia
anemia
hepatosplenomegalia
bone lesions
mucocutaneousmanifestations
(n = 28)
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+
Case 21 month 2 weeks, pseudoparalysis of Parrot
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
Bone lesions with superimposed fractures
Case 31 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
Case 42 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+
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
Case 61 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+
Treatment of the Newborn
Maternal Rx
Clinical Findings in Newborn
Drug(Penicillin G)
Route
None orinadequate
Present Aqueousor
Procaine
IM/IV
IM
10-14 days
None orinadequate
Absent Aqueousor
Procaineor
Benzathine
IM/IVIMIM
10-14 days
Adequate (during
pregnancy)
Absent Benzathine (CDC)or
Follow-up only (AAP)
IM Single dose
Adequate (before
pregnancy)
Absent Follow-up only Or
Benzathine IMSingle dose
Remington & Klein, 2006
Post-treatment Follow-up
Patient Category Follow-up Procedures
Infants - diagnosed as having congenital syphilis
1. 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.
2. Perform treponemal antibody test after age of 15 mo.3. If CNS disease, repeat CSF evaluation every 6 mo until
normal. With abnormal CSF on re-testing, re-treat.4. Careful developmental evaluation, vision and hearing
testing
Infants - who received treatment in utero or at birth because of maternal syphilis
1. RPR testing at birth and then every 3 mo until result is negative.
2. Treponemal antibody test after age of 15 mo.
Rathbun KC, Sex Transm Dis 10:102, 1983
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”
“Saber shins” “Clutton’s joints”
Hutchinson’s teeth
Remington, 2006
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
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