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Congenital Syphilis – in Theory and in Reality in Estonia, 1991-2005 Liis Toome Tallinn Childrens Hospital 15.09.2006.

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Presentation on theme: "Congenital Syphilis – in Theory and in Reality in Estonia, 1991-2005 Liis Toome Tallinn Childrens Hospital 15.09.2006."— Presentation transcript:

1 Congenital Syphilis – in Theory and in Reality in Estonia, Liis Toome Tallinn Childrens Hospital

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

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 Childrens Hospital K. Kink West Tallinn Central Hospital E. Tamm Childrens Clinic, Tartu University Hospitals A. Kangur North Estonia Regional Hospital

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

5 The Incidence of Congenital Syphilis in Estonia, 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 on darkfield Treponema Pallidum

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 Wimbergers 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, n = 28 Tallinn - 12 cases, Harjumaa - 7 cases, Lääne-Virumaa - 5 cases

12 Childrens Age at the Time of Diagnosis in Estonia, (n = 28)

13 Clinical Manifestations of Congenital Syphilis in Estonia, (n = 28)

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 BW 2900 g 1 month unexplained rhinitis anemia Hgb 72 g/l, ER 2,5x10 12 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

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 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 days None or inadequate Absent Aqueous or Procaine or Benzathine IM/IV IM days Adequate (during pregnancy) Absent Benzathine (CDC) or Follow-up only (AAP) IMSingle dose Adequate (before pregnancy) Absent Follow-up only Or Benzathine IM Single dose Remington & Klein, 2006

21 Post-treatment Follow-up Patient CategoryFollow-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

22 Late Congenital Syphilis - after the first two years of the life Dentition Hutchinsons teeth Eye interstitial keratitis Ear eighth nerve deafness Skin, face rhagades, saddle nose CNS mental retardation, HC Bones and joints saber shins, Cluttons joints Saber shins Cluttons joints Hutchinsons teeth 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

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