Congenital syphilis

Background

Epidemiology

• Rare, with about 20 per 100,000 live births in the US
• Greatest risk to child is with untreated primary maternal syphilis

Pathophysiology

• Transplacental transmission while bacteremic
• Can be transmitted during delivery, as well

Clinical Presentation

• Mothers typically have had no prenatal care
• To the fetus, can cause spontaneous abortion (40% in untreated primary syphilis), preterm delivery, polyhydramnios, intra-uterine growth restriction, hydrops fetalis, or intrauterine fetal demise
• At birth, two thirds of affected neonates are asymptomatic, with disease developing over the following 6 weeks
• Early disease, within the first two years, includes:
  • Rhinitis (called snuffles, often bloody and copious), desquamating rash, hepatosplenomegaly, lymphadenopathy, and skeletal abnormalities
  • Also: condyloma lata, vesicular or bullous rash, periostitis, hydrops, thrombocytopenia, hepatitis, jaundice, or glomerulonephritis
  • About 20% involve the CNS
• Late disease, after the first two years, includes:
  • Sensorineural hearing loss, intellectual impairment, saddle nose deformity, frontal bossing, jaw, dental, and palatal abnormalities including Hutchison teeth, saber tibia, short stature, and keratitis

Diagnosis

• Darkfield microscopy and/or PCR on body fluids, including nasal discharge or CSF
• Serology
  • RPR on infant blood (not cord blood), paired with maternal RPR
  • May need CSF analysis
• Also check HIV serology, skeletal survey, chest x-ray, ophthalmology, audiology, and cranial ultrasound

Management

• Treat syphilis in pregnancy with high-dose penicillin to prevent congenital syphilis
• Treat affected infant with penicillin G 50,000 U/kg/day IV q12h for the first 7 days of life, followed by q8h to complete a total of 10 days