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CONGENITAL
SYPHILIS
Sachin Sabu
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)
INCIDENCE
• Occurrence of cong. syphilis – indication of STD in a
given population
• Sub-SaharanAfrica- 10% pregnant women are affected by
syphilis
• Decline in incidence after the introduction of penincillin
• In India - <1/1000 of childhood STD’s
CLASSIFICATION
Congenital
syphilis
Early
Direct bacterial
infection
Within first 2 years
Late
Phenomenon of
hypersensitivity
Later than 2 yrs
Early congenital syphilis
Early cong.
syphilis
• Primary stage – absent (blood borne)
• Baby – Preterm/full term
Lack
manifestations -
birth
rhinitis,
pneumonia,
failure to thrive
Classical
presentation -
birth
Wizened, pot
belly, hoarse baby
looking like old
man, withered
brown skin &
runny fissured
nose.
More prone for
intercurrent
infection
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.
Multiple, discrete, tense
blisters seen over a
normal looking skin
Contain serous/
seropurulent discharge
(spirochetes)
• Condylomata lata- flat topped, hypertrophic, moist
papules which are greyish white.
 Present- angle of mouth, nose, perianal, vulvar.
• 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 alopecia
Mucous membrane lesions
Smooth
greyish
white
mucous
patch
Palate
Tongue
Buccal
Nasal
Genital
Pharynx
Larynx
• 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
• Throat lesions :pharigitis & obstruction of larynx occurs
characteristic hoarse cry (syphilitic apnoea)
Lymph Nodes
• Generalized lymphadenopathy seen in 50% of the cases.
• Nodes are multiple , discrete & non tender
• Epitrochlear lymphnodes are considered pathognomonic
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.
Eyes
• Choroidoretinits, glaucoma, uveitis .
• Choroidoretinitis in later life is seen as salt & pepper
fundus showing black pigment & white atrophic patches.
Centralnervous system
Asymptomatic
No clinical disease
Abnormal CSF
findings
Symptomatic
Meningeal or
meningoencephalitis
involvement
Convulsions, bulging
fontanelles, stiffness of
neck, hydrocephalus &
CSF findings
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
Late congenital syphilis
Stigmata
• They are scars & deformity resulting from cong. Syphilis
• Few are characteristic & remain as permanent evidence of
infection. Eg:
1. “Hot cross bun” look of the cranium.
bossing due to chondritis & focal osteitis)
2. Olympian brow
3. Saddle nose
4. Short maxilla
5. High arched palate
(frontal & parietal
1. “Bull dog jaw” (prominent mandible)
2. “ Sabre tibia”
3. Scaphoid shape of the scapula
4. “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
Hutchinson’striad
Hutchinsons
teeth
Interstitial
keratitis
Neural
deafness
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
Neuraldeafness
• 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)
Nervoussystem
• Clinical manifestations may be symptomatic /
asymptomatic
• 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
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
‘Higoumenakis sign’
• Dactilitis – rarely occurs.
third of the clavicle
Clutton’sjoint
• 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
• Occasionally elbow joint is involved
Other organs
• Liver is occasionally involved
• Cardiovascular syphilis is quite rare
Paroxysmal cold
haemoglobinuria
• Present in both congenital & acquired syphilis
• Due to the presence of thermolabile haemolysin in blood
• This test can be performed in vitro as a diagnostic test.
This
antibody
sensitizes
RBC
Hemolyses
them in the
presence of
complement
Donath
Landsteiner
reaction
• C/F:
Malaise
Headache
Back pain
Fever
Urticaria
“Coca cola “coloured urine , clears in 1-2 days
• Antisyphilitic treatment cures the condition & prevents
further attacks.
Diagnosis
1. Demonstration of T. pallidum by direct examination –
nasal discharge/ early lesions of congenital syphilis.
1. A positive non-treponemal test in a titre higher than the
mother / rising titre in serial monthly tests.
• (but these results do not necessarily indicate infection of the
infant & may be due to the presence of reagin & specific
antibodies which has passed from the maternal to fetal
circulation)
2. An active infection can be ruled out by performing
FTA– ABS test
3. Western blot supplementing FTA-ABS tests on serum
4. PCR on CSF fluid.
Recommended Evaluation
• CSF analysis for VDRL , cell count and protein
• CBC , differential & platelet count
• Other tests as clinically indicated ( long bone radiographs,
chest radiograph, liver function tests, cranial ultasound,
opthamologic examination and auditory brainstem
response)
Treatment
Recommended Regimens
1. Aqueous crystalline penincillin G-
• 1,00,000 – 1,50,000 units/kg/day
• Administered as 50,000 units/kg/dose IV
 First 7 days- 12th hourly
 Next 10 days – 8th hourly
OR
1. Procaine penincillin G-
• 5,00,000 units/kg/dose IM in a single daily dose – 10days
• If more than 1 day therapy is missed , the entire course
should be restarted.
Evaluation & treatment of older infants
and children
• Any child at risk for congenital syphilis should receive a
full evaluation and testing for HIV infection.
• Recommended evaluation:
CSF analysis for VDRL , cell count and protein
CBC , differential & platelet count
Other tests as clinically indicated ( long bone radiographs,
chest radiograph, liver function tests, cranial ultasound,
opthamologic examination and auditory brainstem response)
• Recommended regimen:
1. Aquoeus crystalline penincillin G –
• 2,00,000 – 3,00,000 units/kg/day IV
• Every 4 to 6 hours- 50,000 units/kg
2. If child has no clinical symptoms and CSF is normal,
CSF VDRL is negative –
• 50,000 units/kg IM upto 3 weekly doses.
Thankyou

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congenitalsyphilis-140810152305-phpapp02 (2).pptx

  • 2. 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)
  • 3. INCIDENCE • Occurrence of cong. syphilis – indication of STD in a given population • Sub-SaharanAfrica- 10% pregnant women are affected by syphilis • Decline in incidence after the introduction of penincillin • In India - <1/1000 of childhood STD’s
  • 4. CLASSIFICATION Congenital syphilis Early Direct bacterial infection Within first 2 years Late Phenomenon of hypersensitivity Later than 2 yrs
  • 5. Early congenital syphilis Early cong. syphilis • Primary stage – absent (blood borne) • Baby – Preterm/full term Lack manifestations - birth rhinitis, pneumonia, failure to thrive Classical presentation - birth Wizened, pot belly, hoarse baby looking like old man, withered brown skin & runny fissured nose. More prone for intercurrent infection
  • 6. 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.
  • 7. Multiple, discrete, tense blisters seen over a normal looking skin Contain serous/ seropurulent discharge (spirochetes)
  • 8.
  • 9.
  • 10. • Condylomata lata- flat topped, hypertrophic, moist papules which are greyish white.  Present- angle of mouth, nose, perianal, vulvar. • 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 alopecia
  • 11.
  • 12.
  • 14. • 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
  • 15. • Throat lesions :pharigitis & obstruction of larynx occurs characteristic hoarse cry (syphilitic apnoea)
  • 16. Lymph Nodes • Generalized lymphadenopathy seen in 50% of the cases. • Nodes are multiple , discrete & non tender • Epitrochlear lymphnodes are considered pathognomonic
  • 17. 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.
  • 18.
  • 19.
  • 20. Eyes • Choroidoretinits, glaucoma, uveitis . • Choroidoretinitis in later life is seen as salt & pepper fundus showing black pigment & white atrophic patches.
  • 21.
  • 22. Centralnervous system Asymptomatic No clinical disease Abnormal CSF findings Symptomatic Meningeal or meningoencephalitis involvement Convulsions, bulging fontanelles, stiffness of neck, hydrocephalus & CSF findings
  • 23. 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
  • 25. Stigmata • They are scars & deformity resulting from cong. Syphilis • Few are characteristic & remain as permanent evidence of infection. Eg: 1. “Hot cross bun” look of the cranium. bossing due to chondritis & focal osteitis) 2. Olympian brow 3. Saddle nose 4. Short maxilla 5. High arched palate (frontal & parietal 1. “Bull dog jaw” (prominent mandible) 2. “ Sabre tibia” 3. Scaphoid shape of the scapula 4. “Higoumenakis’ sign” – thickening of the medial third of clavicle
  • 26.
  • 27.
  • 28.
  • 29. • 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
  • 30.
  • 31. • 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
  • 33. 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
  • 34. Neuraldeafness • 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)
  • 35. Nervoussystem • Clinical manifestations may be symptomatic / asymptomatic • Juvenile paresis is more common than juvenile tabes • Dementia may occur • Ass. with optic atrophy
  • 36. 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
  • 37. Bone lesions • Gummas may involve long & flat bones • Manifest as diffuse / localized 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 ‘Higoumenakis sign’ • Dactilitis – rarely occurs. third of the clavicle
  • 38. Clutton’sjoint • 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 • Occasionally elbow joint is involved
  • 39.
  • 40. Other organs • Liver is occasionally involved • Cardiovascular syphilis is quite rare
  • 41. Paroxysmal cold haemoglobinuria • Present in both congenital & acquired syphilis • Due to the presence of thermolabile haemolysin in blood • This test can be performed in vitro as a diagnostic test. This antibody sensitizes RBC Hemolyses them in the presence of complement Donath Landsteiner reaction
  • 42. • C/F: Malaise Headache Back pain Fever Urticaria “Coca cola “coloured urine , clears in 1-2 days • Antisyphilitic treatment cures the condition & prevents further attacks.
  • 43. Diagnosis 1. Demonstration of T. pallidum by direct examination – nasal discharge/ early lesions of congenital syphilis. 1. A positive non-treponemal test in a titre higher than the mother / rising titre in serial monthly tests. • (but these results do not necessarily indicate infection of the infant & may be due to the presence of reagin & specific antibodies which has passed from the maternal to fetal circulation) 2. An active infection can be ruled out by performing FTA– ABS test 3. Western blot supplementing FTA-ABS tests on serum 4. PCR on CSF fluid.
  • 44. Recommended Evaluation • CSF analysis for VDRL , cell count and protein • CBC , differential & platelet count • Other tests as clinically indicated ( long bone radiographs, chest radiograph, liver function tests, cranial ultasound, opthamologic examination and auditory brainstem response)
  • 46. Recommended Regimens 1. Aqueous crystalline penincillin G- • 1,00,000 – 1,50,000 units/kg/day • Administered as 50,000 units/kg/dose IV  First 7 days- 12th hourly  Next 10 days – 8th hourly OR 1. Procaine penincillin G- • 5,00,000 units/kg/dose IM in a single daily dose – 10days • If more than 1 day therapy is missed , the entire course should be restarted.
  • 47. Evaluation & treatment of older infants and children • Any child at risk for congenital syphilis should receive a full evaluation and testing for HIV infection. • Recommended evaluation: CSF analysis for VDRL , cell count and protein CBC , differential & platelet count Other tests as clinically indicated ( long bone radiographs, chest radiograph, liver function tests, cranial ultasound, opthamologic examination and auditory brainstem response)
  • 48. • Recommended regimen: 1. Aquoeus crystalline penincillin G – • 2,00,000 – 3,00,000 units/kg/day IV • Every 4 to 6 hours- 50,000 units/kg 2. If child has no clinical symptoms and CSF is normal, CSF VDRL is negative – • 50,000 units/kg IM upto 3 weekly doses.