Dx & Mx of CONGENITAL SYPHILIS
Prof Ashwani K Sood
Dx & Mx of CONGENITAL SYPHILIS
• Syphilis is a chronic systemic, sexually or vertically
transmitted
• Easily treatable, if detected early
• Significant morbidity, if left unchecked
MICROBE..
Treponema pallidum, a spirochete
Sub species : pallidum
endemicum
carateum
Needs dark field ,phase contrast microscopy or direct
immunoflurescent or silver staining or PCR
• 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
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
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
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
• 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.
• 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.
• Blood and moist open lesions from infants with
congenital syphilis are infectious until 24 hr of
appropriate treatment
• 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.
• 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
• 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
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
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
• 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).
• 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
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.
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.

Diagnosis & Mx of CONGENITAL SYPHILIS.pptx

  • 1.
    Dx & Mxof CONGENITAL SYPHILIS
  • 2.
    Prof Ashwani KSood Dx & Mx of CONGENITAL SYPHILIS
  • 3.
    • Syphilis isa chronic systemic, sexually or vertically transmitted • Easily treatable, if detected early • Significant morbidity, if left unchecked
  • 4.
    MICROBE.. Treponema pallidum, aspirochete Sub species : pallidum endemicum carateum Needs dark field ,phase contrast microscopy or direct immunoflurescent or silver staining or PCR
  • 5.
    • 2 formsof syphilis can occur in children and adolescents * Congenital * Acquired - Almost exclusively by sexual contact - Less commonly via contaminated blood or direct contact
  • 6.
    CONGENITAL SYPHILIS • Transplacentaltransmission 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
  • 7.
    Maternal factors influencingacquisition 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
  • 8.
    CLINICAL FEATURES • Majorityare 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
  • 9.
    • All stagesof 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.
  • 10.
    • Coombs-negative hemolyticanemia 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.
  • 11.
    • Blood andmoist open lesions from infants with congenital syphilis are infectious until 24 hr of appropriate treatment
  • 12.
    • Bone involvementis 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.
  • 13.
    • Congenital neurosyphilis :oftenasymptomatic 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
  • 14.
    • 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
  • 17.
    Late manifestations ofcongenital syphilis • Hypersensitivity phenomena. - Clutton joint - U/L or B/L interstitial keratitis,choroiditis, retinitis - vascular occlusion, optic atrophy - Soft-tissue gummas, - paroxysmal cold hemoglobinuria
  • 18.
    DIAGNOSIS • Results mustalways 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
  • 19.
    • 2-step screeningprocess 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).
  • 20.
    • In congenitalinfection, 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
  • 21.
    Treponemal tests - Toconfirm 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.
  • 22.
    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.