Congenital Syphilis in Theory and in Reality in Estonia, 19912005

1
Congenital Syphilis in Theory and in Reality
in Estonia, 1991-2005

• Liis Toome
• Tallinn Childrens Hospital
• 15.09.2006

2
Incidence of Syphilis in 2001 per 100 000
Population
Estonia 2005 8,2/100 000
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, 1991-2001(-2005)
• Methods
• retrospective analysis of the cases of CS in
  children aged lt 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, 1971 - 2001
Number of cases
Health Protection Inspectorate,
2006
5
The Incidence of Congenital Syphilisin Estonia,
1982 - 2005
0 - 14 years
lt 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
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
11
Congenital Syphilis in Estonia, 1991-2005
n 28
Tallinn - 12 cases, Harjumaa - 7 cases,
Lääne-Virumaa - 5 cases
12
Childrens Age at the Time of Diagnosisin
Estonia, 1991-2005
(n 28)
13
Clinical Manifestations of Congenital Syphilis
in Estonia, 1991-2005
(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 21 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 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

17
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

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 1640

19
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

20
Treatment of the Newborn
Remington Klein, 2006
21
Post-treatment Follow-up
Rathbun KC, Sex Transm Dis 10102, 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

Hutchinsons teeth
Saber shins
Cluttons 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