Congenital syphilis

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Congenital syphilis
Hutchinson teeth congenital syphilis PHIL 2385.rsh.jpg
Notched incisors known as Hutchinson's teeth which are characteristic of congenital syphilis
Causes Born to a mother with syphilis
Medication Penicillin
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Congenital syphilis is syphilis present in utero and at birth, and occurs when a child is born to a mother with syphilis.[1] Untreated early syphilis infections results in a high risk of poor pregnancy outcomes, including saddle nose, lower extremity abnormalities, miscarriages, premature births, stillbirths, or death in newborns. Some infants with congenital syphilis have symptoms at birth, but many develop symptoms later. Symptoms may include rash, fever, an enlarged liver and spleen, and skeletal abnormalities.[1] Newborns will typically not develop a primary syphilitic chancre but may present with signs of secondary syphilis (i.e. generalized body rash). Often these babies will develop syphilitic rhinitis ("snuffles"), the mucus from which is laden with the T. pallidum bacterium, and therefore highly infectious. If a baby with congenital syphilis is not treated early, damage to the bones, teeth, eyes, ears, and brain can occur.[1]

Classification

Early

File:The face of a newborn infant with Congenital Syphilis.tif
The face of a newborn infant displaying snuffles indicative of congenital syphilis
File:Diseases of children (1916) (14597432110).jpg
"Hereditary" syphilis: radiating fissures of the lips (1916).

This is a subset of cases of congenital syphilis. Newborns may be asymptomatic and are only identified on routine prenatal screening. If not identified and treated, these newborns develop poor feeding and runny nose.[1] By definition, early congenital syphilis occurs in children between 0 and 2 years old. After, they can develop late congenital syphilis.[2]

Late

File:Portrait of Gerard de Lairesse MET rl1975.1.140.R.jpg
Gérard de Lairesse, Dutch Baroque painter who suffered from congenital syphilis.
File:Syphilis face 1.jpg
Severe facial disfigurement caused by congenital syphilis, 1880

Late congenital syphilis is a subset of cases of congenital syphilis. By definition, it occurs in children at or greater than 2 years of age who acquired the infection trans-placentally.[2]

Symptoms include:[3]

  • Blunted upper incisor teeth known as Hutchinson's teeth
  • Deafness from auditory nerve disease
  • Frontal bossing (prominence of the brow ridge)
  • Hard palate defect
  • Inflammation of the cornea known as interstitial keratitis
  • Protruding mandible
  • Saber shins
  • Saddle nose (collapse of the bony part of nose)
  • Short maxillae
  • Swollen knees

A frequently-found group of symptoms is Hutchinson's triad, which consists of Hutchinson's teeth (notched incisors), keratitis and deafness and occurs in 63% of cases.[3]

Treatment (with penicillin) before the development of late symptoms is essential.[4]

Signs and symptoms

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Death from congenital syphilis is usually due to bleeding into the lungs.

Diagnosis

Serological testing is carried out on the mother and the infant. If the neonatal IgG antibody titres are significantly higher than the mother's, then congenital syphilis can be confirmed. Specific IgM in the infant is another method of confirmation. CSF pleocytosis, raised CSF protein level and positive CSF serology suggest neurosyphilis.[8]

Treatment

If a pregnant mother is identified as being infected with syphilis, treatment can effectively prevent congenital syphilis from developing in the fetus, especially if she is treated before the sixteenth week of pregnancy. The fetus is at greatest risk of contracting syphilis when the mother is in the early stages of infection, but the disease can be passed at any point during pregnancy, even during delivery (if the child had not already contracted it). A woman in the secondary stage of syphilis decreases her fetus's risk of developing congenital syphilis by 98% if she receives treatment before the last month of pregnancy.[9] An affected child can be treated using antibiotics much like an adult; however, any developmental symptoms are likely to be permanent.[10]

Kassowitz's law is an empirical observation used in context of congenital syphilis stating that the greater the duration between the infection of the mother and conception, the better the outcome for the infant. Features of a better outcome include less chance of stillbirth and of developing congenital syphilis.[11]

The Centers for Disease Control and Prevention recommends treating symptomatic or babies born to an infected mother with unknown treatment status with procaine penicillin G, 50,000 U/kg dose IM a day in a single dose for 10 days.[12] Treatment for these babies can vary on a case by case basis. Treatment cannot reverse any deformities, brain, or permanent tissue damage that has already occurred.[10]

A Cochrane review found that antibiotics may be effective for serological cure but in general the evidence around the effectiveness of antibiotics for congenital syphilis is uncertain due to the poor methodological quality of the small number of trials that have been conducted.[13]

References

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External links

Classification
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External resources

Media related to Lua error in package.lua at line 80: module 'strict' not found. at Wikimedia Commons