Congenital syphilis (CS)


Severe, disabling infection that occurs due to the transmission of Treponema pallidum across the placenta during pregnancy or from contact with an infectious genital lesion during delivery.

History:

Reported cases of early symptomatic congenital syphilis in Canada from 1984 to 1997 per 100,000 population. | Health Canada Sexually transmitted diseases in Canada: 1996 surveillance report (with preliminary 1997 data) Can Commun Dis Rep. 1999;25:S1.
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  • Risk of mother-to-child transmission (MTCT):
    • Highest in primary & secondary stages (60% risk) followed by early latent syphilis
    • However, transmission risk continues during the first 4 years after exposure, after which vertical transmission risk declines over time

Clinical features

Maternal manifestations:

  • Stillbirth (late second and third-trimester fetal death) or death shortly after delivery (30% cases)
  • Spontaneous abortion
  • Non-immune hydrops
  • IUGR
  • Enlarged & pale placenta

Diagnosis of congenital syphilis can be difficult because more than two-thirds of affected infants are asymptomatic at birth, and signs of symptomatic infants may be nonspecific or subtle.

Early congenital syphilis:

Clinical manifestations before 2 years of age
  • Cutaneous lesions:
    • Symmetrical, copper-red maculopapular rash (M/C cutaneous finding of early congenital syphilis)
    • Other findings: Acral skin desquamation, acral vesiculobullae (pemphigus syphiliticus), mucous patches, petechiae, erythema multiforme-like targetoid lesions, perioral/perinasal/perianal fissures, and condylomata lata
  • Snuffles (increased secretions containing spirochetes)
  • Jaundice
  • Hepatomegaly ± splenomegaly
  • Fever
  • Generalized lymphadenopathy
  • Failure to thrive
  • Systemic involvement:
    • Coombs-negative hemolytic anemia
    • Thrombocytopenia
    • Neurosyphilis
    • Pneumonia
    • Hepatitis
  • Skeletal abnormalities:
    • Wimberger sign: Lucencies (demineralization) and erosions (osseous destruction) of the proximal medial tibial metaphysis
    • Wegner sign: metaphyseal lucent bands, metaphyseal serrated appearance at the epiphyseal margin of long bones
    • “Moth-eaten” appearance: diaphyseal periostitis, irregular areas of increased density and rarefaction
    • Pseudoparalysis of Parrot: Multiple painful osteochondritis, resulting in the refusal to move the involved limb
Clinical findings related to CS in children younger than 1 year | Phiske M. M. (2014). Current trends in congenital syphilis. Indian journal of sexually transmitted diseases and AIDS, 35(1), 12–20. https://doi.org/10.4103/0253-7184.132404

Late congenital syphilis:

Clinical manifestations after 2 years of age (usually manifesting around puberty)
  • Orofacial manifestations:
    • Rhagades: Perioral fissures
    • Saddle nose deformity
    • Frontal bossing
    • Hutchinson’s triad:
      1. Peg-shaped, notched, widely spaced permanent upper central incisors
      2. Interstitial keratitis
      3. CN VIII deafness
    • Mulberry molars: multicusped first molars
    • Mental retardation
    • Perforation of the hard palate
    • Prognathism (protrusion of mandible)
  • Skeletal manifestations:
    • Clutton joints: Painless effusion of knees
    • Higoumenakis sign: Thickening of sternoclavicular joint
    • Scaphoid scapula
    • Saber shins: Anterior bowing of shins
Clinical findings related to CS in children older than 1 year | Phiske M. M. (2014). Current trends in congenital syphilis. Indian journal of sexually transmitted diseases and AIDS, 35(1), 12–20. https://doi.org/10.4103/0253-7184.132404

Diagnosis

Algorithm for the treatment and diagnosis of congenital syphilis. | *Fluorescent Treponemal Antibody-Absorption or Microhemagglutination Assay-Treponema Pallidum. †Consider to be serofast only if Venereal Disease Research Laboratory/Rapid Plasma Reagin (RPR) is 1:4. CSF Cerebrospinal fluid | Arnold, S. R., & Ford-Jones, E. L. (2000). Congenital syphilis: A guide to diagnosis and management. Paediatrics & child health, 5(8), 463–469. https://doi.org/10.1093/pch/5.8.463

Management

Treatment protocol | Phiske M. M. (2014). Current trends in congenital syphilis. Indian journal of sexually transmitted diseases and AIDS, 35(1), 12–20. https://doi.org/10.4103/0253-7184.132404
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