this is small effort to summarize congenital syphilis

1. CONGENITAL
SYPHILIS
 Dr. Usha Chandra

2. SYPHILIS
 Sexually transmitted disease.
 Treponema pallidum subspecies pallidum.
 Gram negative.
 5-20 micrometer in length, .10 to .18
micrometer in diameter, regular coil length of
1.1 micrometer.

3. Congenital syphilis
 Infection of fetus in utero as a result of
syphilitic infected mother and refers to all
outcome of pregnancy ( spontaneous
abortion, still birth, live syphilitic child).
 Incidence varies place to place.
 Incidence depend on prevalence of
infectious syphilis in population.

4.  Untreated primary or secondary syphilis
25% risk of stillbirth, 14% risk of neonatal
death, 41% risk of giving birth to a infected
infant and 20% chance of giving birth to
uninfected infant.
 Untreated late syphilis
12% risk of stillbirth, 9% risk of neonatal
death, 2% risk of giving birth to infected
infant, and 77% chance of giving birth to an
uninfected infant.

5. Classification
Congenital
syphilis
Early
Direct bacterial
infection
Within first 2 years
Late
Phenomenon of
hypersensitivity
Later than 2 yrs

6. Early congenital syphilis

7. Pathology
 Early gestational syphilis (<20 weeks) –
not appreciated
 Late gestational syphilis-
Fetal  small perivascular inflammatory foci &
lymphocytic infiltrate  reduced growth of
parenchymal cells & fibrosis
Placental Necrotizing funisitis
Villous enlargement
Acute villitis

8.  Stillborn fetus-
macerated
appearance with
haemorrhagic bulla,
collapse of skull,
protuberant
abdomen with
enlarged liver and
spleen,

9. Early cong.
syphilis
Lack
manifestations -
birth
rhinitis,
pneumonia, failure
to thrive
Classical
presentation – birth
Marasmic syphilis
Wizened, pot belly,
hoarse baby
looking like old
man, withered
brown skin &
runny fissured
nose.
More prone for
intercurrent
infection

10. Skin Lesions
 Vesicobullous lesion, symetrically
on palms and soles- earliest &
specific sign.(Pemphigus
syphiliticus)
 Lesions are contagious, also seen
around oral cavity, trunk, buttocks,
and genitalia.
 Few weeks later, papulosquamous
rash may appear. Usually involve
the face, mouth, anterior nares,
buttocks, palms & soles.

11. Skin Lesions
Rhagades-
healed linear scar of
radiating fissures
produced due to
movement of lips.
Condylomata lata-
Flat topped, greyish white,
hypertrophic, moist papules
angle of mouth, nose,
perianal, vulvar

12. Mucous membrane lesions
Smooth
greyish
white
mucous
patch
Palate
Tongue
Buccal
NasalGenital
Pharynx
Larynx
(aphonia)

13. smooth greyish white patch
watery nasal discharge
(snuffles)
thick, purulent & bloody
discharge
breathing and suckling
difficulties
ulceration & perforation of
nasal septum
saddle nose

14.  Lymph Nodes-
Generalized lymphadenopathy (50%).
Nodes are multiple, discrete & non tender.
Epitrochlear lymphnodes (20%)
(pathognomonic).
 Nail- syphilitic paronychia (due to nail bed
involvement)  atrophic nail, claw nail
deformity.
 Hair-brittle and sparse patchy alopecia

15. Bone lesions
 During first six months – osteochondritis of long bones
(upper end of tibia, distal end of radius & ulna)
 Assymptomatic / severe pain, tenderness while handling
with consequent loss of movements  syphilitic
pseudo paralysis.
 Syphilitic dactilitis- painless fusiform swellings of the
digits, osteochondritis of phalanges occur in the second
year of life.

16. Bone lesions
Decreased mineralization of
the metaphyses of long
bones of the upper
extremities
localized bony destruction
of the medial portion of the
proximal tibial metaphysic
(Wimberger’s sign)

17. Eyes
 Choroidoretinits, glaucoma,
uveitis .
 Choroidoretinitis in later life
is seen as salt & pepper
fundus showing black
pigment & white atrophic
patches.

18. Central nervous system
 Asymptomatic
 No clinical disease
 Abnormal CSF findings
 Symptomatic
 Meningeal or meningoencephalitis involvement
 Convulsions, bulging fontanelles, stiffness of neck,
hydrocephalus & CSF findings

19. Other organ systems
 Liver & spleen – hepatosplenomegaly & ascites 
protuberant abdomen, associated with jaundice &
hypoproteinaemia.
 Kidneys- hyaline , albumin & granular casts in urine.
Proliferative / membraneous glomerulonephritis may be seen.
 Lungs- infiltration of lungs is known as ‘white pneumonia
or pneumonia alba’.
 Pancreas & intestines – syphilitic diarrhoea
 Heart- myocarditis

20. Late congenital syphilis
 Beyond 2 year of life
 Due to hypersensitivity.
 80% go unnoticed in early phase.
 It correspond to tertiary phase of
congenital syphilis.

21. Skin & mucous membrane lesions
 Gummas – nodules,
nodulo ulcerative &
subcutaneous lesions.
 nasal septal & palatal
perforation( nasal twang
& regurgitation of food)
 Leucomelanoderma of
palm( india)

22. Stigmata
 scars & deformities, characteristic & remain as
permanent evidence of infection.
“Hot cross bun” look of the cranium. (frontal & parietal
bossing due to chondritis & focal osteitis)
Olympian brow bony prominence of forehead.
Saddle nose , Short maxilla, High arched palate, “Bull
dog jaw” (prominent mandible)

23. Stigmata
1. “ Sabre tibia”
thickening of middle
third cause anterior
bowing
2. Scaphoid shape of
the scapula
3. “Higoumenakis’
sign” – thickening of
the medial third of
clavicle

24. Hutchinson’s triad
• Hutchinsons teeth
• Interstitial keratitis
• Neural deafness( cochlear part of 8th nerve.)

25. Interstitial keratitis
 It’s the most common
late manifestation of
syphilis
 Age : 5 – 15yrs.
 Symptoms : unilateral
photophobia, pain,
excessive watering of
eyes & blurred vision.
 Usually starts in one eye,
the other eye is likely to
be involved.

26. Circumcorneal vascularization
Salmon patch
(dull pink patch at corneal periphery)
Vascular infiltration extending
from sclera
Cellular exudation
Syphilitic nebula
(corneal ground glass
appearance)

27. Hutchinsons’ teeth
 Seen at 6yrs / later.
 Permanent short ,
upper central
incisors.
 Widely spaced
 Have a notch.
 Assume a peg / cork
screw driver shape.
 Due to defective
enamel formation
 Other incisors may
also be effected

28. Mulberry / Moon’s molars:
 First lower molars –
commonly effected
 Under developed & poorly
enameled
 Bitting surface - dome
shaped with small
projections of ill developed
cusps
 More prone to caries
 Usually lost in early life

29. Nervous system
 Symptomatic / asymptomatic
 Juvenile paresis (common) than juvenile tabes
 Dementia,optic atrophy may occur.
Bone lesions
 Gummas may involve long & flat bones,
OSTEOPERIOSTITIS.
 Bones- thickened , tender
 Sabre tibia ,Parrot nodes, Higoumenakis sign’

30. Clutton’s joint
 Perisynovitis of the knee
joint (3%),elbow.
 Age: 8 – 15yrs
 Hydroarthrosis--painless
swelling, insidious in onset
& chronic in course, usually
bilateral.
 Mobility is preserved.
 X-ray –enlargement of joint
spaces with no bone change

31. Paroxysmal cold haemoglobinuria
 In congenital & acquired syphilis.
 Due to the presence of thermolabile haemolysin in
blood.
 This antibody sensitizes RBC during period of chilling,
then hemolyses them in the presence of
complement when body temperature became
normal-Donath Landsteiner reaction.
 This test can be performed in vitro as a diagnostic
test.

32. Manifestation. malaise, headache, back
pain, fever, urticaria ,Coca cola coloured
urine (clears in 1-2 days).
 Antisyphilitic treatment cures the condition &
prevents further attacks.

33. Diagnosis
1. Direct examination for demonstration – nasal
discharge/ early lesions of congenital syphilis.
2. A positive non-treponemal test in a titre higher
than the mother / rising titre in serial monthly
tests.
(but it may be due to the presence of reagin & specific
antibodies which has passed from the maternal to
fetal circulation)
3. An active infection FTA – ABS test
4. Western blot supplementing FTA- ABS .
5. PCR on CSF fluid.

34. Live borne infant with infected mother
Physical examination,2ml of venous blood of infant and
mother for qualitative and quantitative RPR and VDRL ,
Symptomatic infant
Or
Infant serum quantitative RPR titre is 4 x higher than mother’s titre
Treat infant with with procaine penicillin G 50,000 units/kg IM daily for 10 days
Aqueous crystalline penicillin G 50,000 units/kg IV every 12hr for first 7 days and
8 hrly for next 3 days (total for 10 days)

35. Infant is assymtomatic
And
Either infant serum quantitative RPR titre is <4X higher than
mother’s titre or titre not known
Mother adequately treated
during pregnancy prior to 4
weeks of delivery with
benzathine penicillin
Mother not/inadequately
treated during pregnancy < 4
weeks before delivery, non
penicillin regimen was used,
status / document unknown
Infant with benzathin
penicillin G 50,000
units/kg im
Treat infant with with procaine penicillin G
50,000 units/kg IM daily for 10 days
Aqueous crystalline penicillin G 50,000
units/kg IV every 12hr for first 7 days and
8 hrly for next 3 days (total for 10 days)
g

36.  If more than 1 day of therapy is missed,
the entire course should be restarted.
Data are insufficient regarding the use of
other antimicrobial agents (e.g.,
ampicillin).

37. FOLLOW UP
 All neonates with reactive non treponemal tests
should follow up every 2–3 months until the test
becomes nonreactive.
 In the neonate who was not treated antibody
titers should decline by age 3 months and be
nonreactive by age 6 months, indicating passive
transfer of maternal IgG antibody.
 Treated neonates that exhibit persistent
nontreponemal test titers by 6–12 months should
be re-evaluated through CSF examination and
managed in consultation with an expert.
Retreatment with a 10-day course of a penicillin G
regimen may be indicated.

38.  Neonates with a negative nontreponemal test
at birth and whose mothers were seroreactive
at delivery should be retested at 3 months.
 Treponemal tests should not be used to
evaluate treatment response because the
results are qualitative and passive transfer of
maternal IgG treponemal antibody might
persist for at least 15 months.
 Neonates whose initial CSF evaluations are
abnormal should undergo a repeat lumbar
puncture ( 6 monthly) until the results are
normal. A reactive CSF VDRL test or abnormal
CSF indices that persist and cannot be
attributed to other ongoing illness requires
retreatment for possible neurosyphilis and
should be managed in consultation with an
expert.

39. Special Considerations
Penicillin Allergy
 desensitized and treated with penicillin.
 Major determinant (benzylpenicilloyl poly-L-
lysine [Pre-Pen]) and penicillin G have been
available commercially.

40.  If penicillin is not available, ceftriaxone can be
considered with careful clinical and serologic
follow-up (as evidence is insufficient)
.Management might include a repeat CSF
examination at age 6 months .
cautionly use in infants with jaundice.
 All neonates with congenital syphilis and HIV
infection should be managed similarly as
neonates with congenital syphilis who do not
have HIV infection.

41. THANK YOU