Congenital

syphilis
MODERATOR :-
BY
DR. M.S.BATAR SIR
DR.RAVI KUMAWAT
JUNIOR RESIDENT
MD SKIN & VD
DEPT OF SKIN & V.D. SK HOSPITAL SIKAR
 Introduction
• Congenital syphilis is 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).
Congenital syphilis can be divided into :-
1. EARLY congenital syphilis:-
• PATHOLOGY:-
• Placenta may show - small perivascular
inflammatory foci & lymphocytic infiltrate
reduced growth of parenchymal cells & fibrosis.
• Placenta- heavy, bulky, pale, greasy.
• Placenta HPE-
1. Necrotizing funisitis
2. Villous enlargement
3. Acute villitis
Stillborn fetus-
macerated appearance with collapse of skull,
protuberant abdomen with enlarged liver and
spleen, haemorrhagic bulla.
 •rhinitis,
Lack
Early cong. manifestations - birth •pneumonia,
syphilis •failure to thrive

Classical •Wizeeing

presentation - birth •potbelly,

•hoarse baby looking like
old man, with red brown
skin &
•runny fissured nose.
•More prone for recurrent
infection
Early congenital syphilis showing serous nasal discharge from
nasopharyngitis – ‘syphilitic snuffles’.
• Skin Lesions:-
– Vesicobullous rash, symetrically , palms and soles earliest &
specific sign.
– Also known as Pemphigus syphiliticus
• Lesions are contagious, also seen around oral cavity, trunk,
buttocks, and genitilia.
• Few weeks later, a papulosquamous rash may appear. Usually
involve the face, mouth, anterior nares, buttocks, palms &
soles.
Rhagades- healed linear scar of radiating fissures produced
due to movement of lips.
• Nail- syphilitic paronychia
(due to nail bed involvement)
atrophic nail, claw nail deformity.
• Hair-brittle and sparse patchy (moth eaten alopecia)
Mucous membrane lesions
• Lead to erosions / snail track ulcers
• In nasal mucosa, smooth greyish white patch
watery nasal discharge (snuffles) thick, purulent &
bloody discharge
• breathing and suckling difficulties
• ulceration & perforation of nasal septum
saddle nose
Bone lesions :-
• During first six months – osteochondritis of long bones
(upp. end of tibia, distal end of radius & ulna)
• Child presents with severe pain, tenderness while
handling with consequent loss of movements syphilitic
pseudo paralysis.
• Wimberger’s sign- loss of density on the medial side
of upper end of tibia .
• Syphilitic dactilitis- painless fusiform swellings
of the digits, osteochondritis of phalanges
occur in the second year of life
Early congenital syphilis showing osteitis of the skull
bones (arrow) causing a worm-eaten appearance.
Wimberger’s sign (arrow) – destruction of the
proximal metaphyses of the tibiae.
Widespread syphilitic osteitis, periostitis and destructive metaphysitits of the
long bones.
Dactylitis of the little finger in late congenital syphilis.
Eyes :-
• Chorio-retinits, glaucoma, uveitis .
• Chorio-retinits in later life is seen as salt &
pepper fundus showing black pigment & white
Atrophy.
• Other organ systems:-
• Liver & spleen – hepatosplenomegaly & ascites
protuberant abdomen. It may be associated with
jaundice & hypoproteinaemia
• Kidneys- presence of 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.
2. Late congenital syphilis:-
• They are scars & deformity resulting from cong. Syphilis
Phenomenon of hypersensitivity Later than 2 yrs
• Few are characteristic & remain as permanent evidence of
infection. Eg:
1. look of the cranium. (frontal & parietal bossing due to chondritis &
focal osteitis)
2. Olympian brow
3. Saddle nose
4. Short maxilla
5. High arched palate
6. “Bull dog jaw” (prominent mandible)
7. “ Sabre tibia”
8. Scaphoid shape of the scapula
9. “Higoumenakis’ sign” – thickening of the medial third of clavicle
Hutchinsons’ teeth
• Seen at 6yrs / later Permanent upper central incisors are
shorter than the lateral incisors Widely spaced Have a notch
in the bitting edge Due to defective enamel formation
• Assume a peg / cork screw driver shape Other incisors may
also be effected
• 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
• 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 in a matter
of 2 weeks
• Neural deafness
• Hypersensitivity reaction to treponemes.
• Due to involvement of cochlear part of VIII nerve
• Symptoms :
– Tinnitis
– Vertigo
– Hearing loss
– Cochlear degeneration (osteochondritis of otic
capsule)
– Sensorineural deafness (ossicles involvment)
• Nervous system:-
– Juvenile paresis is more common than juvenile tabes
– Dementia may occur
– Ass. with optic atrophy
• Skin & mucous membrane lesions
• Gummas – usual presenting features .
• They may manifest as nodules, nodulo ulcerative &
subcutaneous lesions nasal septal & palatal perforation nasal
twang & regurgitation of food
• Bone lesions
• Gummas may involve long & flat bones Manifest as diffuse
/ localized gummatous osteoperiostitis
• Bones- thickened , tender
• Tibia is most frequently involved, thickening of middle third
causes anterior bowing ‘
Sabre tibia’
• Localized osteoperiostitis of the skull bones causes the
formation of rounded, bony swelling Parrot nodes’
• Thickening of the inner third of the clavicle Higoumenakis
sign’
• Dactilitis – rarely occurs
• Clutton’s joint:-
• Perisynovitis of the knee joint Age: 8 – 15yrs
• Leads – hydroarthrosis
It’s a painless swelling, insidious in onset & chronic in course
• Usually B/L knees are involved
• Mobility is preserved (no impairment of function)
• X-ray –enlargement of joint spaces with no bone change
3. Stigmata :-
– Hutchsion teeth
– Mulberry molar
– Hutchsion triad (IK+HT+8TH N.Deafness)
– Ragades,at the corner of the mouth
– saddle nose
– Bull dog jaw
– High arched palate
– Sabre tibia
– Corneal opacities
• Evaluation of neonates for congenital syphilis:-
• Examination for stigmata of congenital syphilis.
• X-ray of long bones for evidence of periostitis.
• CSF examination.
• Dark-field microscopy and/or PCR from exudates of
suspicious lesions or fluids.
• Infection of the neonate is also suggested if the
serum nontreponemal antibody titre is four or more
times more than the mother’s,
• Or if specific IgM treponemal antibody tests are
positive
First line treatment
Scenario 1 Scenario 2
• Infants with proven or highly probable Infants with a normal physical examination and a
disease and one of the following: non-treponemal serological titre the same or
less than fourfold the maternal titre,and one
of the following :-
1. An abnormal physical finding that is 1. The mother was not treated,
consistent with congenital syphilis. inadequately treated or has no
2. A quantitative non-treponemal documentation of having received
serological titre that is fourfold treatment.
higher than the mother’s titre. 2. The mother was treated with
3. positive dark-field test of body erythromycin or another nonpenicillin
fluid(s).
regimen.
3. The mother received treatment <4
The recommended regimens are:
weeks before delivery
• Aqueous crystalline penicillin G
The recommended regimens are:
100 000–150 000 units/kg/day,
Aqueous crystalline penicillin G
administered as 50 000 units/kg/dose IV
every 12 h during the first 7 days of life 100 000–150 000 units/kg/day
and every 8 h thereafter for a total of administered as 50 000 units/kg/dose IV every 12 h
10 days. during the first 7 days of life
and every 8 h thereafter for a total of 10 days.
First line treatment
Scenario 3 Scenario 4
• Infants with a normal physical • Infants with a normal physical
examination and non-treponemal examination and non-treponemal
serological titre the same or less serological titre the same or less
than fourfold the maternal titre than fourfold the maternal titre
and both of the following: and both of the following:
1. The mother was treated during 1. The mother’s treatment was adequate
pregnancy, treatment was appropriate before pregnancy.
for the stage of infection, and 2. The mother’s non-treponemal serological
treatment was administered >4 weeks titre remained low and stable before and
before delivery. during pregnancy and at delivery
2. The mother has no evidence of (VDRL<1 : 2; RPR <1 : 4).
reinfection or relapse.
The recommended regimens are:
The recommended regimens are: • Benzathine penicilline
• Benzathine penicilline administered as 50,000 units/kg/dose.
administered as 50,000 IM in a single dose.
units/kg/dose. IM in a single dose
• Scenario 5
In older infants and children the
recommended regimen is
Aqueous crystalline penicillin G 2,00,000–
3,00,000 units/kg/day IV,
administered as 50 000 units/kg every 4–6 h
for 10 days.
• Second line:-
• penicillin allergy then ceftriaxone can be used.
However, if a non-penicillin agent is used, close
serological and CSF follow-up are indicated.
• For infants aged ≥30 days:
ceftriaxone 75 mg/kg IV/IM per day
in a single daily dose for 10–14 days.

• For older infants: ceftriaxone 100 mg/kg per day

in a single dose.
•Thank you