Title: CONGENITAL SYPHILIS
1CONGENITAL SYPHILIS
2- Syphilis is a chronic infection caused by the
spirochete T. pallidum, which is of particular
concern during pregnancy because of the risk of
transplacental infection of the fetus - Congenital infection is associated with severe
adverse outcomes - -perinatal death
- -premature delivery
- -Low birth weigth
- -congenital anomalies
3Modes of transmission
- Sexual contact
- Transplacental, from the mother to the fetus
- By contact with a lesion at the time of delivery
- The risk of developing syphilis after contact is
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4Risk factors for CS
- Lack or inadequate PNC
- Maternal substance abuse
- Failure to repeat a serologic test for syphilis
during the third trimester - Treatment failure
- Inadequate access to STDs clinics and outreach
activities
5Epidemiology
The CS rate peaked in 1991 at 107.3 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
6CDC surveillance
- 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.
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8Maternal treatment history among 451 infants with
CS in US in 2002
9PATHOGENESIS/PATHOLOGY
10Syphilis in newborns
- Congenital syphilis
- - Transplacental
- beginning 9 - 10 weeks
- analogous to secondary adquired syphilis
- affects bone, brain, liver, lung
- placenta large and thickened, hypercellular
villi, UC abscess-like necrotic foci - - Vertical transmission
- more freq. primary and secondary dz.
- Risk diminishes with after 4 years of infection
11Syphilis in newborns
- 2/3 of NB with CS are asymptomatic at birth
- Overt infection can manifest in the fetus, the
newborn or late childhood - The infant may have many or even no signs until
6-8 wks of life (delayed form) - Clinical manifestations after birth are divided
in - -early CS
- -late CS 2 yo
12Clinical manifestations of early CS
- 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.
13- Secondary lesions on face they first appeared
during the fourth postnatal week.
14- 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
15- Other stigmata seen before the age of 2 years
include maculopapular rash, hepatosplenomegaly
and jaundice.
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17 Congenital syphilis. Diaphysitis with abundant
callus formation secondary to pathologic
fractures through the metaphyseal lesions. The
lesions healed, and there were no sequelae
18Clinical manifestations of late onset
- Hutchinsons triad (63)
- Hutchinson teeth (blunted upper incisors)
- Interstitial keratitis
- VII nerve deafness
- Frontal bossae (bony prominence of the forehead)
83 - Saddle nose 74
- Defect of hard pallate
- Cluttons joint (bilateral painless swelling of
the knees) - Saber chins
- Short maxillas
- Protruding mandible
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21Laboratory diagnosis
- Direct visualization
- Serologic testing
-
22Serologic Testing
- The non-treponemal screening tests include the
VDRL (Venereal Disease Research Laboratory), RPR
(rapid plasma reagin), or ART (automated reagin
test). - - usually correlate with dz activity, in titers
- - On the other hand, other disease states or
physiologic states, such as pregnancy, can yield
false-positive results. -
23Serologic Testing
- 2. Treponemal-specific tests including
fluorescent treponemal antibody absorption test
(FTA-ABS) or Treponema pallidum particle
agglutination (TP-PA) are necessary to confirm
the diagnosis of syphilis after a positive
nontreponemal test. - Nontreponemal screening during pregnancy is
recommended at the first prenatal visit, and
again in the third trimester, particularly in
high-risk populations
24- Adapted from Sexually Transmitted Diseases, by
Holmes, Sparling, Mardh, et al.
25Case definitions for CS
- CONFIRMED OR DEFINITE CS
- a. infant lesions
- b. placenta
- c. ummbilical cord
- d. amniotic fluid
- e. autopsy material
-
26Case definitions for CS
- 2. PRESUMPTIVE OR PROBABLE,
- if mother had
- a. untreated syphilis
- b. no documentation of treatment
- c. non penicillin therapy
- d. penicillin
- if baby has a reactive treponemal test and any
of - a. any evidence of CS on clinical exam
- b. any evidence of CS on long bone radiograph
- c. positive VDRL in CSF
- d. abnormal CSF without any other cause
- e. quantitative nontreponemal test titer 4 fold
higher than maternal titer - f. reactive treponemal antibody test beyond 15
months.
27Case definitions for CS
- 3. POSSIBLE in asymptomatic infants when
-
- a. reactive treponemal or nontreponemal test
- b. maternal tx during pregnancy s/
post-treatment fall in titers - c. maternal tx before pregnancy s/ adequate
follow up - infants whose mothers were treated1mo before
delivery AND had a documented fourfold decline in
titers AND have no evidence of reinfection or
relapse, are UNLIKELY to have the infection. -
28Evaluation of neonates with Suspected or
Confirmed CS
- Detailed physical examination
- Quantitative nontrep. Test on infant
- Specimens for testing for the presence of
spirochetes form mucocutaneous lesions - CBC to assess for anemia or thrombocytopenia
- CSF analysis
- Long bone radiographs unless the diagnosis has
been confirmed otherwise - Pathologic examination of the placenta or UC.
29Treatment
- Definitive or probable CS
- IV aqueous crystalline penicillin G x 10-14 days
50 000UI/kg q 12hr (1-7 dol) and q 8hr (8-30dol) - IM procaine penicillin G 50 000U/kg/dose q day
for 10 to 14 days
30Treatment
- 2. Infants with probable syphilis, BUT who are
asymptomatic and c/ normal evaluation - If f/u is certain a single dose IM benzathine
penicillin G may be adequate - Some experts will prefer a 10-14 day full course
if any part of the evaluation is abnormal or
uninterpretable. - 3. Asymp. Infants with possible CS.
- a) single dose of benzathine penicillin
31Follow up
- Re-evaluation after treatment at 1, 2, 3, 6 and
12 months of age. - Nontreponemal tests should be repeated every 2 to
3 months until they have become nonreactive or
diminished four-fold. - Maternal origin Ab (nontreponemal) titers become
negative within 3 mo, and should become negative
at 6 mo. - Treponemal-specific Ab of maternal origin persist
for 12 to 15 mo IN 15 of uninfected infants from
seropositive mothers. - Congenital neurosyphilis should be evaluated
(clinical and CSF) every 6 months until CSF
clears.
32Follow up evaluation
- Non treponemal antibody serologic testing should
be checked at 1,3,6, 12 and 24 months following
the treatmetn - Titers should decrease by four fold by 6 months
of therapy and became non reactive by 12 or 24
months - Titers that show a four fold rise or do not
decrease suggest either failure of treatment or
reinfection
33- Sexually transmitted infections remain a major
public health concern in the - United States. An estimated 19 million infections
occur each year - Sexually transmitted infections are relatively
common during pregnancy, especially - in indigent, urban populations effected by drug
abuse and prostitution. - Education, screening, treatment, and prevention
are important components of - prenatal care for women at increased risk for
these infections. Treatment of these - sexually transmitted infections is clearly
associated with improved pregnancy - outcome and reductions in perinatal mortality
34- Syphilis is caused by the spiroquete Treponema
pallidum. - Syphilis is primarily acquired through sexual
contact,though approximately 1000 cases of
vertically acquired congenital infections occur
each year in the United States. - Antepartum syphilis can profoundly affect
pregnancy outcome by causing - preterm labor, fetal death, and neonatal
infection by transplacental or perinatal - infection 8,9. Fortunately, of the many
congenital infections, syphilis is not - only the most readily prevented but also the most
susceptible to therapy.
35DIAGNOSIS
- Diagnostic testing involves a two-step process,
beginning with a nonspecific test and concluding
with a treponeme-specific test for patients
screening positive. - The non-treponemal screening tests include the
VDRL (Venereal Disease Research Laboratory), RPR
(rapid plasma reagin), or ART (automated reagin
test). Nontreponemal test antibody titers usually
correlate with disease activity and should be
reported with a quantitative titer. - On the other hand, other disease states or
physiologic states, such as pregnancy, can yield
false-positive results. - Because the current incidence of syphilis is so
low, the majority of positive screening tests are
not due to treponemal infection. - Treponemal-specific tests including fluorescent
treponemal antibody absorption test (FTA-ABS) or
Treponema pallidum particle agglutination (TP-PA)
are necessary to confirm the diagnosis of
syphilis after a positive nontreponemal test.
These tests are specific for T pallidum antigens
and are reported as positive or negative. - Nontreponemal screening during pregnancy is
recommended at the first prenatal visit, and
again in the third trimester, particularly in
high-risk populations
36TREATMENT
- Penicillin G, in benzathine, aqueous procaine, or
aqueous crystalline form, is the drug of choice
for treatment of all stages of syphilis, and is
the only effective treatment for the prevention
of congenital syphilis in pregnancy. - Erythromycin may be curative in the mother, but
may not prevent congenital syphilis because of
the variability of transplacental passage of the
antibiotic. - Ceftriaxone may prove useful in adults as an
alternative regimen for patients who have
penicillin allergy however, there is
insufficient information on its use in pregnancy - The efficacy of azithromycin in the
penicillin-allergic pregnant woman has not been
adequately evaluated.
37- A recommended dosage regimen for pregnant women
is as follows - Primary, secondary, or early latent stage
benzathine penicillin G, 2.4 million units
intramuscularly (IM) in a single dose - Late latent stage or syphilis of unknown
duration benzathine penicillin G, 2.4 million
units IM once a week for 3 consecutive weeks - Neurosyphilis aqueous crystalline penicillin G,
34 million units intravenously (IV) every 4
hours, or 1824 million units daily as continuous
infusion, for 1014 days - The rate of treatment failure may be increased in
pregnant patients who have secondary syphilis,
therefore some experts recommend the use of a
second injection of benzathine penicillin G 2.4
million units IM 1 week after the first to treat
early syphilis in pregnancy
38- Within hours after treatment, patients can
develop an acute complication called the
Jarisch-Herxheimer reaction. Symptoms include
fever, chills, myalgias, headache, tachycardia,
hyperventilation, vasodilation, and mild
hypotension. Uterine contractions and fetal heart
rate decelerations may occur. - Although the reaction occurs in 10 to 25 of
patients overall, it is most common in the
treatment of early syphilis. A recent report 20
noted an incidence of 40 among treated pregnant
women. Symptoms last for 12 to 24 hours and are
usually self-limiting. Patients can be treated
symptomatically with antipyretics. Routine
hospitalization is not recommended for treatment
during pregnancy, though this has not been
systematically evaluated 16.
39Evaluation of treatment
- Consideration should be given to ultrasound
evaluation of the fetus before - therapy when syphilis is diagnosed after 24
weeks. Ultrasound abnormalities - associated with syphilis include polyhydramnios,
hepatosplenomegaly, ascites, - and hydrops 21. Fetuses that have physical
evidence of severe disease discovered - on ultrasound have also been shown to have
biochemical evidence - of severe disease. Treatment failure and other
complications are more common - among these fetuses 22. When the fetal
ultrasound is abnormal, consultation - with specialists in maternal-fetal medicine and
neonatology should occur. Complications - such as preterm labor, preterm premature rupture
of the membranes, - fetal heart rate decelerations, and stillbirth
may be precipitated by treatment. - In the severely affected fetus, particularly with
preexisting fetal heart rate - abnormalities, consideration may be given to an
untreated preterm or term - delivery followed by neonatal treatment 16,23.
Despite the advantages of - ultrasound assessment, maternal treatment should
not be delayed unduly to obtain - imaging.