Congenital syphilis is a severe infection transmitted from mother to fetus that can cause stillbirth or life-threatening complications in infants. Without treatment, half of infected fetuses die in utero or shortly after birth from complications like pneumonia or bleeding in the lungs. It is diagnosed through tests detecting the syphilis bacterium or antibodies in the infant's blood or spinal fluid. Treatment with intravenous or intramuscular penicillin is highly effective but must be given to the newborn if the mother was not adequately treated during pregnancy or is at high risk of transmitting the infection. With proper treatment, outcomes are good but untreated infants may develop late complications affecting the bones, teeth, eyes or brain.

1. Congenital
syphilis

2. Congenital syphilis
• Severe, disabling, and often life-threatening
infection seen in infants
• About half of all infected fetuses die shortly
before or after birth

3. Pathophysiology CS
• Trans placental transmission
• Transmission rate:~ 60 - 100%
• With early onset disease, manifestations
result from trans placental spirochetemia and
are analogous to secondary stage of acquired
syphilis
• CS does not have a primary stage

4. Clinical Manifestations
• Intra-uterine: -Placenta
-Fetus
• Post-natal: - Early
- Late

5. Intra-uterine: Placenta
• The placenta is typically large and edematous
• Characteristic placental findings include:
- Hydrops placentalis
- Chronic villitis
- Perivillous fibrous proliferation
- Normoblastemia
- Necrotizing funisitis
- Acute chorioamnionitis
- Plasma cell deciduitis

6. Intra-uterine: Fetus
• Depends on stage of development at time of
infection & duration of untreated infection
• Initially characterized by placental
involvement and hepatic dysfunction (e.g.,
abnormal LFT), followed by amniotic fluid
infection, hematologic abnormalities, ascites,
and hydrops
• Stillbirth / Neonatal death

7. Intra-uterine: Fetus
• >24 weeks gestation: 66 % of fetuses have
either congenital syphilis or T.Pallidum
detected in amniotic fluid
• Intrauterine death: 25 % of affected
• Perinatal mortality: 25-30 %, if untreated

8. Post-Natal
• Among survivors, manifestations been divided into:
Early stage = First 2 years
Late stage = After 2 years
• Inflammatory changes do not occur in the fetus until
after first trimester → organogenesis is unaffected
• Nevertheless, all organ systems may be involved

9. Early SIGNS & SYMPTOMS-
Asymptomatic
• Occurs between 0 - 2 years
• If asymptomatic :
- Identified on routine prenatal screening
- If not identified and treated, these
newborns develop poor feeding and
rhinorrhea
➨ Earliest signs of CS may be poor feeding
and snuffles (i.e., syphilitic rhinitis)

10. Symptomatic Early SIGNS &
SYMPTOMS-
If Symptomatic:
• Variable
• Appear within 1st 5 weeks of life
• Stillborn/ Premature
• Failure to gain weight or FTT
• Fever / Irritability
• Severe congenital pneumonia

11. Symptomatic Early SIGNS &
SYMPTOMS-
• Most striking lesions affect the
mucocutaneous tissues and bones:
- Mucous patches
- Rhinitis =snuffle
- Condylomatous lesions
➨ ➨ highly characteristic features of mucous
membrane involvement in CS

12. Symptomatic Early SIGNS &
SYMPTOMS-
• Snuffles → Followed quickly by diffuse
maculopapular desquamative rash that
involves extensive sloughing of the epithelium,
on the palms & soles and around the mouth &
anus
• When chronic → “Saddle Nose”
• Lesions & nasal fluid: highly infectious

13. Symptomatic Early CS
• Bullous skin disease known as “pemphigus
syphiliticus”
➲ Early rash -- small blisters on the palms
and soles → Ulcerated
➲ Later rash -- copper-colored, flat or
bumpy rash on the face, palms, and soles

14. Symptomatic Early CS
• Other early manifestations include
hepatosplenomegaly (100%), jaundice, anemia
• Metaphyseal dystrophy and periostitis often
are noted on radiographs at birth +/_
Pseudoparalysis

15. Congenital syphilis - early evidence of infection
- bullae and vesicular rash

16. Multiple, punched out, pale, blistered lesions, with
associated desquamation of palms & plantars

17. Intraoral mucous patches & facial skin lesions

18. Secondary lesions on feet
Lesions first appeared during 4th week

19. Late-onset CS
• Develop from scarring related to early
infection
• Can be prevented by treatment within first 3
months
• Can appear as late as 40 years after

20. Late-onset CS
• Manifestations include neurosyphilis and
involvement of teeth, bones, eyes, and 8th
cranial nerve
• E.g.: Frontal bossing, short maxilla, high
palatal arch, Hutchinson triad, saddle nose,
and perioral fissure (Rhagades = bacterial
infection of skin lesions )

21. Hutchinson triad
1. Deafness (10 – 40 years)
2. Hutchinson’s teeth = centrally notched,
widely-spaced peg-shaped upper central
incisors
3. Interstitial Keratitis → blindness (5-20
years)

22. Notched incisors known as Hutchinson’s teeth

23. Moribund newborn with CS
Oral / skin lesions and saddle nose

24. Metaphyseal osteomyelitis
Radiolucent distal radius & ulna with cupping distal ulna

25. Osteochondritis of femur & tibia

26. 1-m-old . Classical Wimberger's sign of destructive metaphysitis
involving medial aspects of distal femora and proximal tibae

27. “Saber shins” = Osteoperiostitis Tibia

28. Interstitial keratitis

29. Possible Complications
• Blindness
• Deafness
• Facial deformity
• Neurological problems

30. Labs
Definitive diagnosis:
1. By direct visualization of spirochetes using dark
field microscopy
2. Or direct fluorescent antibody tests of lesion
exudate or tissue (Placenta/UC)
-Helpful early in the disease, prior to development of seroreactivity

31. Serologic tests
- Presumptive diagnosis can be made using
- Nontreponemal ( False + in medical conditions)
- Treponemal (False+ in other spirochetal
Diseases)
→ So use of only one type is insufficient
- If nontreponemal test is +→ confirmatory testing is
performed with a specific treponemal test

32. Nontreponemal test
• VDRL (Venereal Disease Research
Laboratory)
• RPR (Rapid plasma reagin)
• ART (Automated reagin test)

33. Nontreponemal test
- Used for screening (sensitive but not specific)
- Inexpensive, performed rapidly, and provide
quantitative results
→ helpful indicators of disease activity & monitor
treatment response
- Measures Ab directed against lipoidal Ag from T.
Pallidum, Ab interaction with host tissues or both
- Nonspecific Ab develop 4-8 weeks following
infection

34. Nontreponemal test
• False negative
- Early primary S
- Latent acquired S
- Late CS
- Prozone phenomenon
• False Positive
- Viral infection ( EBV,
Hepatitis, Varicela,
Measles)
- Lymphoma
- TB
- Malaria
- Endocarditis
- CT diseases
- Pregnancy
- IV drugs
- Wharton Jelly
contamination in cord
samples

35. Nontreponemal test
• Any reactive NT test must be confirmed by
Treponemal test to exclude false positive
• Treatment should not be delayed if symptomatic or
at high risk of infection
• Monitor:
- Sustained 4 fold ↓NT test titer after treatment
→ Adequate treatment
- Sustained ↑: Re-infection or relapse

36. Nontreponemal test
Newborn Dilemma
• Testing of newborn often is problematic
because IgG antibody may be a reflection of
maternal rather than infant infection
• Unless NT titer is much higher in baby than in
mother → f/u serology over 1st 6 months of life,
when maternal IgG is lost, would be required to
make a diagnosis
i.e. Loosing precious time in treatment initiation

37. Treponemal Specific Test
• T pallidum immobilization (TPI)
• Fluorescent treponemal antibody
absorption (FTA-ABS)
• Microhemagglutination assay for antibodies
to T pallidum (MHA-TP)

38. Treponemal Specific Test
• Confirm + nontreponemal reaginic test
• Remain positive for life
i.e. Result do not correlate with disease activity
and tests are not quantified
• False + reactions:
→ Other spirochetal diseases (e.g., yaws, pinta,
leptospirosis, rat-bite fever, relapsing fever,
Lyme disease

39. Cerebrospinal Fluid Analysis
• CSF VDRL
• Could be negative and still develop signs of
neurosyphilis →Therefore, all those with
presumptive CS should be treated
• A nonquantitative VDRL test is the only
serologic test that should be performed on
CSF
Other test like FTA-ABS are less specific on CSF samples

40. CBC
• CS characterized by anemia,
thrombocytopenia, and either leukopenia
or leukocytosis
• Evidence of Coombs-negative hemolytic
anemia or a leukemoid reaction may be
present

41. Imaging Studies
• CXR:
- Syphilitic pneumonia is common in CS
- Fluffy diffuse infiltrate “pneumonia alba”

42. Imaging Studies
• Long bone radiography
– 95% of symptomatic infants and 20% of
asymptomatic
– Multiple sites of osteochondritis at wrists,
elbows, ankles and knees and periostitis of long
bones
– The lower extremities almost always affected

43. Imaging Studies
• Neuroradiography:
- Findings nonspecific
- May mimic herpes simplex virus
- MRI may reveal cerebral hypertrophy
and hyperintensity in the temporal
lobes

44. CDC
Newborn Evaluation
• The diagnosis of CS is complicated by the
trans placental transfer of maternal
nontreponemal and treponemal IgG Abs to
fetus
➨ Making interpretation of reactive serologic
tests for CS difficult

45. CDC
Newborn Evaluation
Evaluation should include:
1. Maternal H/O syphilis including tx type &
adequacy before and during the pregnancy
2. P/E of newborn
3. Quantitative NT & T tests
4. CBC, long bone x-rays, CSF (VDRL, cell count,
protein), and CXR and/or LFT
5. Pathologic examination of placenta or umbilical
cord using specific fluorescent antitreponemal
antibody staining

46. CDC
Newborn Evaluation
• A presumptive diagnosis, which results in tx, is
made if baby has + serologic test
and any of following:
1. Compatible findings on P/E
2. CSF abn. (+ VDRL, ↑ WBC, or ↑protein)
3. Osteitis on x-ray long bones
4. Placentitis
5. NT test 4x > than maternal
6. Positive FTA-ABS-19S IgM antibody

47. Treatment
• IV Penicillin G is the drug of choice for all
stages of syphilis including CS
• Infants:
- 100,000 - 150,000 U/kg/d IV Q12 x 7 d. then Q
8 to complete 10 days
- Or Procaine Penicillin G 50,000 U/kg/d IM once
for 10 days (adequate CSF conc. may not be
achieved)

48. Treatment
• Indications:
1. If newborn meets any of criteria
2. If mother was treated < 4 weeks prior to
delivery
3. If mother treated with other than penicillin
4. If maternal titers suggest inadequate response
to treatment before or early in pregnancy

49. Syphilis In Pregnancy
• In communities in which risk for CS is high →
serologic testing and a sexual history also should
be obtained at 28 weeks gestation and at delivery
• Treat all pregnant patients with penicillin,
regardless of the stage of pregnancy

50. Syphilis In Pregnancy
• 3 doses of benzathine penicillin
(2.4 million U IM at 1-week intervals)
• No proven alternative treatment for patient allergic
to penicillin
i.e. Erythromycin for patient allergic to penicillin is
not reliable treatment for fetus

51. Evaluation and Treatment of Infants During the
First Month of Life
The following scenarios describe the
evaluation and treatment of infants for
congenital syphilis

52. Scenario 1
Infants with proven or highly probable disease
and
• Abnormal P/E consistent with CS
• Serum quantitative NT titer 4x >
mother’s titer or
• + darkfield or fl. ab. test of body fluids

53. Scenario 1
Infants with proven or highly probable disease
and
Recommended Evaluation
• CSF analysis for VDRL,
cell count & protein
• CBC w. diff.& PL count
• Other tests as clinically
indicated ( long-bone x-
rays, CXR, LFT, HUS,
ophthalmologic exam, and
BAER)
Recommended Regimens
• Aqueous crystalline
penicillin G
50,000 U/kg/dose IV Q
12 hrs. first 7 DOL and Q
8 hrs thereafter for a
total of 10 days
OR
• Procaine penicillin G
50,000 units/kg/dose IM
in a single daily dose for
10 days

54. Scenario 2
Normal P/E & serum quantitive NT titer ≤ 4 x maternal titer
• Mother not / inadequately treated, or no
documentation
• Mother was treated with erythromycin
or other nonpenicillin regimen or
• Mother received treatment < 4 weeks
before delivery

55. Scenario 2
Normal P/E & serum quantitive NT titer ≤ 4 x maternal titer
Recommended
Evaluation
• CSF analysis for
VDRL, cell count, and
protein
• CBC w. diff. and PLT
count
• Long-bone X-rays
Recommended
Regimens
• Aqueous cryst. penicillin G
50,000 u./kg/dose IV Q 12 hrs
during the 1st 7 DOL and Q 8
hrs thereafter for a total of 10
days
OR
• Procaine penicillin G 50,000
units/kg/dose IM in a single
daily dose for 10 days
OR
• Benzathine penicillin G 50,000
units/kg/dose IM in a single
dose

56. Scenario 3
Normal P/E & serum quantitive NT titer ≤ 4 x maternal titer
• Mother was treated during pregnancy, tx. was
appropriate for the stage of infection, and
treatment was administered > 4 weeks before
delivery….. and
• Mother has no evidence of reinfection or
relapse

57. Scenario 3
Normal P/E & serum quantitive NT titer ≤ 4 x maternal titer
Recommended
Evaluation
⇩
No evaluation
required
Recommended
Regimen
⇩
Benzathine penicillin G
50,000 units/kg/dose
IM in a single dose

58. Scenario 4
Normal P/E & serum quantitive NT titer ≤ 4 x maternal titer
• Mother’s treatment was adequate before
pregnancy…. and
• Mother’s NT titer remained low and stable
before, during pregnancy and at delivery (VDRL
<1:2; RPR <1:4)

59. Scenario 4
Normal P/E & serum quantitive NT titer ≤ 4 x maternal titer
Recommended
Evaluation
⇩
No evaluation required
Recommended
Regimen
⇩
No treatment
required

60. Outlook (Prognosis)
• Infected early in pregnancy ➨ stillborn
• Treatment of expectant mother ↓ risk of CS
• Babies who become infected when passing through
birth canal have better outlook
• Death from CS is usually through pulmonary
hemorrhage