Congenital syphilis

Congenital syphilis
Notched incisors known as Hutchinson's teeth which are characteristic of congenital syphilis
Usual onset	Congenital
Duration	Lifelong
Types	Early & late
Causes	Born to a mother with syphilis
Diagnostic method	Signs, symptoms, blood tests, CSF tests
Prevention	Adequate screening and treatment in pregnant mother
Treatment	Antibiotic
Medication	Penicillin

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

Signs and symptoms

• 
  13-day-old infant with cleft lip
• 
  The face of a newborn infant displaying snuffles indicative of congenital syphilis
• 
  "Hereditary" syphilis: radiating fissures of the lips (1916).
• 
  Gérard de Lairesse, Dutch Baroque painter who suffered from congenital syphilis.

• Abnormal x-rays
• Anemia^[5]{
• Du Bois sign, narrowing of the little finger
• Enlarged liver
• Enlarged spleen
• Frontal bossing
• Higouménakis' sign, enlargement of the sternal end of clavicle in late congenital syphilis
• Hutchinson's triad, a set of symptoms consisting of deafness, Hutchinson's teeth (centrally notched, widely spaced peg-shaped upper central incisors), and interstitial keratitis (IK), an inflammation of the cornea which can lead to corneal scarring and potential blindness
• Jaundice
• Lymph node enlargement
• Mulberry molars (permanent first molars with multiple poorly developed cusps)^[6]
• Petechiae
• Poorly developed maxillae
• Pseudoparalysis
• Rhagades, linear scars at the angles of the mouth and nose result from bacterial infection of skin lesions
• Snuffles, aka "syphilitic rhinitis", which appears similar to the rhinitis of the common cold, except it is more severe, lasts longer, often involves bloody rhinorrhea, and is often associated with laryngitis^[7]
• Sabre shins
• Skin rash

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

Centers for Disease Control and Prevention infographic about congenital syphilis.

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

External links

• Images of Congenital Syphilis Manifestations Archived 2011-05-22 at the Wayback Machine
• 00655 at CHORUS