Congenital Syphilis

Aka: Congenital Syphilis, Parrot Pseudoparalysis, Neonatal Snuffles, Congenital Syphilitic Pemphigus, Olympian Brow, Syphilitic Saddle Nose, Mulberry Molars, Higoumenakis Sign, Clutton's Joints, Saber Shin

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II. Epidemiology

  1. Congenital Syphilis Incidence in U.S. is increasing
    1. 2012: 7.7 cases per 100,000 live births
    2. 2013: 9.2 cases per 100,000 live births
    3. 2016: 16.2 cases per 100,000 live births
    4. 2017: 23.3 cases per 100,000 live births (918 cases in U.S.)

III. Pathophysiology

  1. Transmitted transplacentally or via contact with syphilitic lesions during delivery

IV. Risk Factors

  1. Children born to mothers who have sex with multiple partners
    1. Unique pocket of Syphilis is in urban women who trade crack Cocaine for sex

V. Signs: Early presentation (birth to 48 months)

  1. Only one third of Congenital Syphilis patients are symptomatic at birth
  2. Common
    1. Hepatosplenomegaly
    2. Morbilliform rash similar to Secondary Syphilis rash
      1. Nickels and Dimes type rash on palms and soles
    3. Fever
    4. Neurosyphilis findings in infants
      1. Bulging Fontanelle
      2. Seizures
      3. Cranial Nerve deficit
  3. Uncommon
    1. Parrot Pseudoparalysis (painful epiphysitis)
      1. Syphilis related bone Growth Plate involvement results in failure to move involved extremities
      2. Rothner (1975) Pediatrics 56(4): 604-5 [PubMed]
    2. Snuffles
      1. Rhinitis characterized by blood stained mucus
    3. Syphilitic Pemphigus
      1. Bullae on distal extremities
  4. Other findings
    1. Anemia
    2. Jaundice
    3. Thrombocytopenia
    4. Reticulocytosis
    5. Edema

VI. Signs: Late presentation (untreated after 48 months to age 20 years)

  1. Common
    1. Olympian Brow (Frontal Bossing)
      1. Frontal bossing or high forehead
    2. Rhagades
      1. Mouth or nose scarring
    3. Saddle nose deformity
      1. Nasal cartilage destruction with depressed Nasal Bridge
    4. Hutchinson's Teeth
      1. Permanent upper central incisors with peg-shape
    5. Mulberry Molars
      1. First molars (6th year molar) with extra cusps (mulberry appearance)
    6. Higoumenakis Sign
      1. Unilateral, proximal clavicular enlargement (near sternoclavicular joint)
    7. Wimberger Sign
      1. Pathognomonic, bilateral symmetric Osteomyelitis
      2. Medial tibia metaphysis pathologic Fractures
  2. Uncommon
    1. Clutton's Joints
      1. Major joint sterile effusions with symmetric distribution
    2. Saber Shins
      1. Tibial bowing
  3. Other Findings
    1. Deafness
    2. Disseminated gummata
    3. Ocular changes
      1. Bilateral interstitial Keratitis
        1. Presents with photophobia, Lacrimation and Corneal vascularization
      2. Chorioretinitis
      3. Optic atrophy

VII. Labs

  1. See Syphilis
  2. Obtain Serologic Non-Treponemal Tests (VDRL or RPR)
    1. Infant and mother at birth
      1. Suspect Congenital Syphilis if infant titer >=4 fold higher titer than mother
    2. Infant every 2-3 months until Non-Treponemal Test nonreactive
  3. Screen for other Sexually Transmitted Infection
    1. See STI Screening

VIII. Evaluation: Active Syphilis Testing in infant

  1. Indications
    1. Findings of Congenital Syphilis on exam
    2. Suspected untreated maternal Syphilis
      1. Mother treated for Syphilis within 4 weeks prior to delivery
  2. Testing
    1. CSF for VDRL, cell count and Protein
    2. Complete Blood Count with Platelets
  3. Diagnostics
    1. Long Bone XRays
    2. Ophthalmology and audiology exams

IX. Management

  1. Precautions
    1. Consider Nonaccidental Trauma and Child Sexual Abuse
    2. Do not miss doses
      1. More than one day of treatment missed requires repeating the entire Antibiotic course
  2. Diagnosis at birth to age 1 month
    1. Penicillin G Benzathine 50,000 U/Kg IM (up to 2.4 million U IM)
      1. Given every 12 hours for 7 days, then every 8 hours for an additional 3 days
    2. Alternatives
      1. Penicillin G Procaine 50,000 U/Kg IM (up to 2.4 million U IM) every 24 hours
      2. Ceftriaxone 75 mg/kg IV/IM every 24 hours for 10-14 days
        1. Risk of worsening Neonatal Jaundice and Kernicterus!
  3. Diagnosis at >1 month of age
    1. Penicillin G Benzathine 50,000 U/Kg IM (up to 2.4 million U IM)
      1. Given every 4-6 hours for 10 days (200-300k units/kg/day)
    2. Alternatives
      1. Penicillin G Procaine 50,000 U/Kg IM (up to 2.4 million U IM) every 24 hours
      2. Ceftriaxone 100 mg/kg IV/IM every 24 hours for 10-14 days

X. Resources

  1. CDC STD management booklet
    1. http://www.cdc.gov/mmwr/pdf/rr/rr5912.pdf

XI. References

  1. (2019) Sanford Guide, accessed on IOS 10/29/2019
  2. Green, Cohen, Billington (2016) Crit Dec Emerg Med 30(11): 4-10
  3. Kirk, McHugh and Parnell (2023) Crit Dec Emerg Med 37(8): 23-9
  4. (2015) MMWR Morb Mortal Wkly Rep 64(RR3):1-37 [PubMed]
  5. Mattel (2012) Am Fam Physician 86(5): 433-40 [PubMed]
  6. Ricco (2020) Am Fam Physician 102(2): 91-8 [PubMed]
  7. Woods (2009) Pediatr Infect Dis J 28(6): 536-7 [PubMed]

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