congenital syphilis

1. Dr Nibin

2. • Syphilis is a chronic systemic, sexually or
vertically transmitted
• Easily treatable, if detected early
• Significant morbidity, if left unchecked

3. MICROBE..
Treponema pallidum, a spirochete
Sub species : pallidum
endemicum
carateum
Needs dark field ,phase contrast microscopy or
direct immunoflurescent or silver staining or PCR

4. • 2 forms of syphilis can occur in children and
adolescents
* Congenital
* Acquired - Almost exclusively by sexual contact
- Less commonly via contaminated
blood or direct contact

5. CONGENITAL SYPHILIS
• Transplacental transmission or occasionally by
intrapartum contact with infectious lesions
( active genital lesions )
• Transmission can occur at any stage of pregnancy
Can result in,
o Early fetal loss
o Preterm births, LBW
o Still births or neonatal deaths ~40%
o Congenital syphilis

6. Maternal factors influencing acquisition of
infection
• Women with primary & secondary syphilis and spirochetemia are
more likely to transmit the disease than those with latent infection
• Highest risk during 1st 4 yrs of infection
• Limited access to health care
• Late or no prenatal care, inadequate Rx during pregnancy
• Inadequate serological response of mother to Rx
• Drug use
• Multiple sex partners, sex trade
• Unprotected sexual contact, incarceration
• Untreated syphilis during pregnancy  ~100 % vertical transmission :
Obliterating endarteritis and profound effects on pregnancy outcome

7. CLINICAL FEATURES
• Majority are asymptomatic @ birth
( including upto 40 % with CSF seeding)
: Identified only by routine prenatal screening
• Without Rx , symptomatic by weeks to months
• Among infants symptomatic at birth or in the first few
months of life, manifestations have traditionally been divided
into,
 Early stage
 Late stage

8. • All stages of congenital syphilis are characterized by a vasculitis, with
progression to necrosis and fibrosis.
• The early signs  Appear during the first 2 yr of life
(resulting from transplacental spirochetemia )
Late signs gradually during the first 2 decades.
• Early manifestations vary and involve multiple organ systems
- Analogous to the secondary stage of acquired syphilis.
- Hepato-splenomegaly, jaundice, and elevated liver enzymes
- Histologically, liver involvement includes bile stasis, fibrosis,
- Extramedullary hematopoiesis.
• Lymphadenopathy tends to be diffuse and resolve spontaneously,
although shotty nodes can persist.

9. • Coombs-negative hemolytic anemia is characteristic.
• Thrombocytopenia is often associated with platelet
trapping in an enlarged spleen.
• Osteochondritis and periostitis : Classic
• Erythematous maculopapular or vesiculobullous rashes
f/b desquamation involving hands and feet
• Mucous patches, persistent rhinitis (snuffles ), and
condylomatous lesions are highly characteristic features
of mucous membrane involvement containing abundant
spirochetes.

10. • Blood and moist open lesions from infants with
congenital syphilis are infectious until 24 hr of
appropriate treatment

11. • Bone involvement is common.
Roentgenographic abnormalities include,
Wimberger lines
Painful osteochondritis at the wrists, elbows, ankles,
and knees with irritability and refusal to move the
involved extremity (Pseudoparalysis of Parrot )
Periostitis of the long bones and rarely the skull.

12. • Congenital neurosyphilis
:often asymptomatic in newborns
• FTT
• Renal involvement include hypertension,
hematuria,proteinuria,hypercholesterolemia, and
hypocomplementemia
(glomerular deposition of circulating immune complexes.)
• Less-common-
gastroenteritis, peritonitis, pancreatitis,
glaucoma,chorioretinitis
• Nonimmune hydrops
• testicular masses
Other manifestations

13. • Late manifestations (children > 2 yr of age)
- rarely seen in developed countries.
- due to chronic granulomatous
inflammation of bone, teeth and CNS
• Skeletal changes – Due to persistent or recurrent
periostitis.
• Dental abnormalities – Hutchinson teeth
Repeated caries.
• Saddle nose

16. Late manifestations of congenital syphilis
• Hypersensitivity phenomena.
- Clutton joint
- U/L or B/L interstitial keratitis,choroiditis, retinitis
- vascular occlusion, optic atrophy
- Soft-tissue gummas,
- paroxysmal cold hemoglobinuria

17. DIAGNOSIS
• Results must always be interpreted in the context of patient
history and physical examination.
• Darkfield microscopy to demonstrate the microbe or direct
fluorescent antibody testing
: specimens from skin lesions, placenta or umbilical cord
• PCR
Despite the absence of a true gold standard serologic assay,
serologic testing for syphilis remains the principal

18. • 2-step screening process
nontreponemal test followed by a confirmatory
treponemal test
• The Venereal Disease Research Laboratory (VDRL) and
rapid plasma reagin (RPR)  Nontreponemal tests
: screening and in monitoring therapy.
• Nontreponemal tests - Usually nonreactive within 1 yr of
adequate therapy for primary syphilis and within 2 yr of
for secondary disease.
• 15–20% of patients become serofast (nontreponemal
titers persisting at low levels for long periods).

19. • In congenital infection, these tests become nonreactive
within a few months after adequate treatment.
• False positive VDRL - IMN and other viral infections,
autoimmune diseases, and pregnancy
• All pregnant women should be screened early in
pregnancy and at delivery.
• False-negative results - Antibody excess
early primary syphilis
latent syphilis of long duration
late congenital syphilis

20. Treponemal tests
- To confirm diagnosis
- Measure specific T. pallidum antibodies (IgG, IgM and
IgA), which appear earlier than nontreponemal ab
• Positive soon after initial infection
• Remain positive for life,
• Do not correlate with disease activity.
T. pallidum particle agglutination test,
T. pallidum hemagglutination assay,
Fluorescent treponemal antibody absorption test.

21. • Interpretation of nontreponemal and treponemal
serologic tests in the newborn can be confounded by
maternal IgG antibodies transferred to the fetus.
• Passively acquired antibody is suggested by a neonatal
titer at least 4-fold (i.e. a 2 tube dilution) less than the
maternal titer.
• Usually undetectable by 3-6 mo of age

22. Neurosyphilis – Difficult diagnosis
Pleocytosis and increased protein in the CSF
Positive CSF VDRL test (specific)
+
Neurologic symptoms.
CSF PCR and IgM immunoblot tests also

23. Placental examination by gross and microscopic
techniques can be useful in the diagnosis of congenital
syphilis. The disproportionately large placentas are
characterized histologically by focal proliferative villitis,
endovascular and perivascular arteritis, and focal or
diffuse immaturity of placental villi.

24. Diagnosis of congenital syphilis
Maternal history of syphilis
Proactive evaluation and treatment of exposed
neonates is critical
Symptomatic infants should be thoroughly
evaluated and treated.

25. • Diagnosis of neurosyphilis in the newborn with syphilitic infection
• poor sensitivity of the CSF VDRL test
• lack of CSF abnormalities.
• . Diagnosis of syphilis beyond early infancy should lead to
consideration of possible child abuse.
• For infants with proven or highly probable disease or abnormal
physical findings, complete evaluation, including serologic tests
(RPR or VDRL), complete blood count with differential and platelet
count, liver function tests, long-bone radiographs, ophthalmology
examination, auditory brainstem response, and other tests as
indicated, should be performed. For infants with a positive VDRL or
RPR test result and normal physical examination whose mothers
were inadequately treated, further evaluation is not necessary if 10
days of parenteral therapy are administered

26. • Treatment The goals of early detection and treatment include treatment of current
infection and prevention of both late stage disease and sexual or vertical transmission. T.
pallidum remains extremely sensitive to penicillin, with no evidence of emerging penicillin
resistance, and thus penicillin remains the treatment drug of choice (Table 245.3 and
http://www.cdc.gov/std/treatment ). Parenteral penicillin G is the only documented
effective treatment for congenital syphilis, syphilis during pregnancy, and neurosyphilis.
Aqueous crystalline penicillin G is preferred over procaine penicillin, because it better
achieves and sustains the minimum concentration of 0.018 µg/mL (0.03 units/mL) needed
for 7-10 days to achieve the prolonged treponemicidal levels required for the long dividing
time of T . pallidum . Although nonpenicillin regimens are available to the penicillinallergic
patient, desensitization followed by standard penicillin therapy is the most reliable
strategy. Success of treatment also depends upon the integrity of the host immune
response. A transient acute systemic febrile reaction called the Jarisch-Herxheimer
reaction (caused by massive release of endotoxin-like antigens during bacterial lysis) occurs
in 15–20% of patients with acquired or congenital syphilis treated with penicillin. It is not
an indication for discontinuing penicillin therapy.

27. • Syphilis in Pregnancy When clinical or serologic findings
suggest active infection or when diagnosis of active syphilis
cannot be excluded with certainty, treatment is indicated. The
goals of treatment of the pregnant woman include eradication
of maternal disease, prevention of mother to child
transmission, and treatment of fetal infection. Patients should
be treated immediately with the penicillin regimen
appropriate for the woman's stage of syphilis. Women who
have been adequately treated in the past do not require
additional therapy unless quantitative serology suggests
evidence of reinfection (4-fold elevation in titer ). Doxycycline
and tetracycline should not be administered during
pregnancy, and macrolides do not effectively prevent fetal
infection. Pregnant patients who are allergic to penicillin
should be desensitized and treated with penicillin.

28. • Adequate maternal treatment at least 30 days prior to delivery is likely to prevent
congenital syphilis. All infants born to mothers with syphilis should be followed until
nontreponemal serology is negative. The infant should be treated if there is any uncertainty
about the adequacy of maternal treatment. The goal of infant treatment is prevention of
organ damage, skeletal deformity, and developmental delay. Any infant at risk of congenital
syphilis should be evaluated for HIV. Congenital syphilis is treated with aqueous penicillin
G (100,000-150,000 units/kg/24 hr divided every 12 hr IV for the 1st wk of life, and every
8 hr thereafter) or procaine penicillin G (50,000 units/kg IM once daily) given for 10 days.
Both penicillin regimens are recognized as adequate therapy for congenital syphilis, but
higher concentrations of penicillin are achieved in the CSF of infants treated with
intravenous aqueous penicillin G than in those treated with intramuscular procaine
penicillin. Treated infants should be followed every 2-3 mo to confirm at least a 4-fold
decrease in nontreponemal titers. Treated infants with congenital neurosyphilis should
undergo clinical and CSF evaluation at 6- mo intervals until CSF is normal. At age 2 these
infants should receive a full developmental assessment. In a very-low-risk neonate who is
asymptomatic and whose mother was treated appropriately, without evidence of relapse or
reinfection, but with a low and stable VDRL titer (serofast), no evaluation is necessary.
Some specialists would treat such an infant with a single dose of benzathine penicillin G
50,000 units/kg IM.

29. • Prevention Syphilis, including congenital syphilis, is a
reportable disease in all 50 states and the District of
Columbia. Testing is indicated at any time for persons
with suspicious lesions, a history of recent sexual
exposure to a person with syphilis, or diagnosis of
another sexually transmitted infection, including HIV
infection. The resurgence of syphilis compels clinicians
to remain cognizant of its protean manifestations to
avoid missed or late diagnosis. Timely treatment lessens
risk of community spread. Despite the genome
sequencing of T. pallidum in 1998, vaccine prevention
remains elusive, confounded by the treponeme's ability
to evade the immune system.

30. • Congenital Syphilis Congenital syphilis is a preventable disease, a
sentinel event indicating multiple missed opportunities. Primary
prevention is tied to prevention of syphilis in women of childbearing
age and secondary prevention with early diagnosis and prompt
treatment of women and their partners. Access to and use of
comprehensive prenatal care is key, with careful history taking
(including interim sexual partners) at each visit. Routine prenatal
screening for syphilis remains the most important factor in
identifying infants at risk for developing congenital syphilis.
Screening all women at the beginning of prenatal care is an
evidence-based standard of care and legally required in all states. In
pregnant women without optimal prenatal care, serologic screening
for syphilis should be performed at the time pregnancy is diagnosed.
Any woman who is delivered of a stillborn infant at 20 wk or fewer
of gestation should be tested for syphilis.

31. • In communities and populations with a high prevalence of syphilis and in
patients at high risk (women with a history of incarceration, drug use, or
multiple or concurrent partners), testing should be performed at least 2
additional times: at the beginning of the 3rd trimester (28 wk) and at
delivery. Some states mandate repeat testing at delivery for all women,
underscoring the importance of preventive screening. Women at high risk
for syphilis should be screened even more frequently, either monthly or
pragmatically in the case of inconsistent prenatal care, at every medical
encounter because they can have repeat infections during pregnancy or
reinfection late in pregnancy. Follow-up serologic testing of all treated
women should be done after treatment to document titer decline, relapse,
or reinfection. No newborn should leave the hospital without the mother's
syphilis status having been determined at least once during pregnancy or at
delivery. In states conducting newborn screening for syphilis, both the
mother's and infant's serologic results should be known before discharge. In
addition, all previously uninvestigated infants of an infected mother should
be screened. Strong linkages between clinicians and public health
practitioners remain essential for comprehensive prevention of acquired
and congenital syphilis.