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Irina Tabidze, MD, MPH and Chicago Dept of Public Health

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1 Irina Tabidze, MD, MPH and Chicago Dept of Public Health
Congenital Syphilis Irina Tabidze, MD, MPH and Thad Zajdowicz, MD, MPH STD/HIV Division Chicago Dept of Public Health Dr Tabidze is an epidemiologist for the STD/HIV Program of CDPH, with a special interest in congenital syphilis and syphilis epidemiology. Dr Zajdowicz is the Medical Director of the STD/HIV Program at CDPH.

2 Congenital Syphilis (CS)
Syphilis is a chronic infection caused by the spirochete Treponema pallidum, which is of particular concern during pregnancy because of the risk of transplacental infection of the fetus. Congenital infection is associated with several adverse outcomes, including: -Perinatal death -Premature delivery -Low birth weight -Congenital anomalies

3 Modes of Transmission:
Sexual contact. Trans-placental passage from infected mother. Contact with lesion at the time of delivery. The risk of developing syphilis after exposure is about 40%. T. pallidum readily crosses the placenta. Vertical transmission can occur at any time during pregnancy and at any stage of the disease. Perinatal transmission occurs in 50% of patients diagnosed with Primary & Secondary (P&S) syphilis, with fewer congenital infections among women with early latent (40%), late latent (10%) and tertiary disease (10%).

4 Risk Factors for CS Lack of or inadequate prenatal care.
Maternal substance abuse. Failure to repeat a serological test for syphilis in the third trimester. Treatment failure. Inadequate access to Sexually Transmitted Diseases (STD) clinics and STD outreach activities. Most congenital cases are due to lack of prenatal care, but a negative test in the first trimester of pregnancy that was not repeated later in pregnancy as well as inadequate maternal treatment are also important contributing factors.

5 Epidemiology of CS Incidence of CS reflects the rate of syphilis in women of childbearing age. Peaks in CS occur one year after peaks in P&S syphilis in women. In the United States, the rate of syphilis in women of childbearing age has fallen in contrast to increases of syphilis rates in men who have sex with men.

6 Congenital syphilis — Rates for infants <1 year of age: US, 1981–2002 and the Healthy People 2010 objective Rate (per 100,000 live births) 125 Cong. Syphilis 2010 Objective 100 75 50 25 The CS rate peaked in 1991 at cases per 100,000 live births, and declined by 90.5% to 10.2 cases/per 100,000 live births in 2002. The HP2010 objective for CS is 1.0 case per 100,000 live births. In 2002, 27 states, the District of Columbia, and one outlying area had rates higher than this objective. Adapted from CDC 1981 83 85 87 89 91 93 95 97 99 2001 Note: The Healthy People 2010 objective for congenital syphilis is 1.0 case per 100,000 live births. The surveillance case definition for congenital syphilis changed in 1988.

7 Congenital syphilis — Reported cases for infants <1 year of age and rates of P &S syphilis among women: US, 1970–2002 P&S rate (per 100,000 population) CS cases (in thousands) 20 7.5 Kaufman Criteria 16 6.0 CDC Surveillance Definition 12 4.5 P&S Syphilis 8 3.0 Congenital 4 1.5 Before 1989 reported cases of CS were defined and classified on the basis of clinical and serological features known as the Kaufman criteria. The Kaufman criteria were not designed for use as a surveillance case definition. In 1988 CDC developed a surveillance case definition for CS. This surveillance case definition differs from the clinical diagnosis of CS in several ways. All infants born to mothers who have untreated or inadequately treated for syphilis are considered potentially infected. Asymptomatic infants and stillbirths are included in the case definition. The surveillance case definition makes case classification simpler. Adapted from CDC Syphilis 0.0 1970 75 80 85 90 95 2000 Note: The surveillance case definition for congenital syphilis changed in 1988.

8 Syphilis in Newborns Two-thirds of live-born neonates with CS are asymptomatic at births. Overt infection can manifest in the fetus, the newborn, or later in childhood. The infant may have many or even no signs until 6-8 weeks of life (delayed form). Clinical manifestations after birth are divided arbitrarily into: - Early CS (<=2 years of age) and - Late CS ( >2 years of age)

9 Clinical Manifestations of Early CS
Condyloma Lata Maculopapular rash Hepatosplenomegaly Jaundice due to the hepatitis Anemia Osteochondritis Snuffles Pseudoparalysis Lymphadenopathy Mucous patches

10 Congenital Syphilis The earliest sign of CS is nasal discharge (snuffles) that occurs 1-2 weeks before the onset of the rash. Treponemes abound in the discharge, providing a definitive means of diagnosis.

11 Secondary lesions on face; they first appeared during the fourth postnatal week.

12 Congenital Syphilis The vesiculobullous eruption, known as pemphigus syphiliticus, is highly distinctive when present. When the bullae rupture, they leave a macerated, dusky red surface that readily dries and crusts.

13 Congenital Syphilis Other stigmata seen before the age of 2 years include maculopapular rash, hepatosplenomegaly and jaundice.

14 Congenital Syphilis

15 Clinical Manifestations of Late CS
Hutchinson’s triad (63%): -Hutchinson’s teeth (blunted upper incisors) -Interstitial keratitis -VII nerve deafness Frontal bossae (bony prominences of the forehead) (87%) Saddle nose (74%) Defect of hard pallet Clutton’s joints (bilateral painless swelling of knees) Saber chins Short maxillas Protruding mandible

16 Congenital Syphilis Hutchinson’s Triad (late congenital syphilis):
Interstitial keratitis Teeth abnormalities Deafness Hutchinson’s triad (blunted upper incisors, interstitial keratitis and VIII nerve deafness) is considered pathognomic of late CS.

17 Congenital Syphilis Classic sign of late congenital syphilis is perforation of the hard palate.

18 Clutton’s Joints

19 Saddle Nose

20 Sabre Shins

21 Laboratory Diagnosis Direct visualization
Darkfield examination of exudate Direct fluorescent antibody to T. pallidum Serologic testing - Nontreponemal Antibody tests (VDRL test and RPR test) - Treponemal Antibody tests (FTA-ABS and MHA-TP) T. pallidum cannot be visualized by light microscopy but can be seen using darkfield microscopy. Serologic testing is an indirect method of diagnosis since it relies upon a maternal humoral immune response to the infection. Two types of serologic testing are available: Nontreponemal Antibody tests (Venereal Disease Research Laboratory(VDRL) and the Rapid Plasma Reagin (RPR)- are performed on serum and used as the screening tests for syphilis. Treponemal Antibody tests (Fluorescent Treponemal Antibody Absorption (FTA-ABS) and microhemagglutination test for antibodies to T. pallidum (MHA-TP) are confirmatory tests that detect antibodies specifically directed at treponemal cellular components.

22 Interpretation of the Syphilis Serology of Mothers & their Infants
Nontreponemal Test Treponemal Test Interpretation No syphilis or incubating syphilis in the mother and infant Mother Infant Mother Infant - - - - + + - - No syphilis in mother Maternal syphilis with possible infant infection + + or - + + Adapted from Sexually Transmitted Diseases, by Holmes, Sparling, Mardh, et al. Recent or previous syphilis in the mother; possible infection in infant + + + + - - + + Mother successfully treated for syphilis before or early in pregnancy;

23 Maternal Treatment Penicillin is the gold standard for the treatment of syphilis. Pregnant women with syphilis should be treated with the appropriate penicillin regimen according to their stage of disease. Sexually Transmitted Diseases Treatment Guidelines MMWR 2002; 51 (No. RR-6): [19-23]

24 Treatment of Infants Sexually Transmitted Diseases Treatment Guidelines MMWR 2002; 51 (No. RR-6): [26-28]

25 Follow-up evaluation Non-treponemal antibody serologic testing should be checked at 1, 3, 6, 12 and 24 months following treatment. Titers should decrease four-fold by 6 months post therapy and become non-reactive by 12 to 24 months. Titers that show a four-fold rise or do not decrease suggest either treatment failure or re-infection.


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