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.
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.
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
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:
Peg-shaped, notched, widely spaced permanent upper central incisors
Interstitial keratitis
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
A photograph of Hutchinson’s teeth resulting from congenital syphilis. Hutchinson’s teeth is a congenital anomaly in which the permanent incisor teeth are narrow and notched. Note the notched edges and “screwdriver” shape of the central incisors. | By CDC/Susan Lindsley – http://phil.cdc.gov/phil_images/20021114/34/PHIL_2385_lores.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=2134349
Mulberry molars are a dental condition usually associated with congenital syphilis, characterized by multiple rounded rudimentary enamel cusps on the permanent first molars.
Sharp anterior bowing, or convexity, of the tibia. | Gonçalves, M. J. and Romão, V. C. (2014) ‘Saber Tibia in Paget’s Disease of Bone’, New England Journal of Medicine. Massachusetts Medical Society, 371(25), p. 2417. doi: 10.1056/NEJMicm1403255.
Photo showing the sclerokeratitis associated with GPA | By Jonathan Trobe, M.D. – University of Michigan Kellogg Eye Center – The Eyes Have It, CC BY 3.0, https://commons.wikimedia.org/w/index.php?curid=16116693
This patient with congenital syphilis shows “Clutton joints,” or symmetrical hydrarthrosis of the knee joint. This is a painless condition that often occurs during the late stages of congenital syphilis. | Reproduced from: the Public Health Image Library, Centers for Disease Control and Prevention. Photo by Susan Lindsley.
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
