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 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 metaphysisWegner 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 rarefactionPseudoparalysis 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 fissuresSaddle 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 molarsMental retardation Perforation of the hard palate Prognathism (protrusion of mandible) Skeletal manifestations:Clutton joints : Painless effusion of kneesHigoumenakis sign : Thickening of sternoclavicular jointScaphoid 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
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