Congenital Syphilis

Congenital Syphilis

Neonates presenting to the Emergency Department often cause a lot of uncertainty. Is this scalp swelling normal? Is this bloody stool concerning? Are this odd movements part of normal development? Is that umbilical area ok or concerning? Fortunately, we have addressed many neonatal issues in the PedEMMorsels. Obviously, one of the most concerning considerations in neonates is the potential for infection. We have previously discussed hypothermia presentations, GBS infections, and HSV infections. There is another significant infection that we need to be aware of presenting in our neonates. Let’s review how Congenital Syphilis may present to our Emergency Departments:

Congenital Syphilis: Basics

  • Occurs when the spirochete Treponema pallidum is transmitted from mother to fetus.
    • Syphilis screening is part of proper prenatal care in the US.
      • Most cases of congenital syphilis occur when there is lack of access to appropriate screening.
      • Additionally, since syphilis is treatable, cases can also occur when appropriate therapy was not received.
    • Congenital syphilis is classified as either Early or Late. [Foles, 2024]
      • Early = symptoms appear within the first 2 years of life (usually within first 3 months)
      • Late = symptoms appear after 2 years of age.

  • Worldwide, congenital syphilis complicates about 1 million pregnancies / year. [Hussain, 2023]
    • Leads to >300,000 perinatal deaths / year globally.
    • Rates of congenital syphilis are increasing. [Foles, 2024]
      • In the US, from 2013-2018, cases increased from 362 to 1,306.
      • 2019, there were 1,875 and this trend has continued to increase (2,157 case in 2020; 2,855 cases in 2021).

  • Congenital syphilis differs from syphilis contracted by an “adult” in that: [Hussain, 2023]
    • T. pallidum invades the bloodstream directly causing spirochetemia in the fetus.
    • This leads to widespread inflammation and involvement of any organ.

  • Syphilis remains a very treatable disease, so early detection is important!
    • Maternal screening early in pregnancy is the best means to prevent congenital syphilis.
    • Penicillin is 98% effective at preventing congenital syphilis. [Hussain, 2023]
    • Excellent prognosis if treated early.
    • If not treated early, then increased risk of poor outcomes and death.
      • Untreated congenital syphilis has high morbidity (~33%) and mortality (~6.5%).

  • One of the ToRCHeS Infections
    • Congenital Syphilis may present similarly as other important congenital infections:
    • Toxoplasmosis
    • Rubella
    • CMV
    • Herpes Simplex Viruses (HIV also)
    • Syphilis

Congenital Syphilis: Presentations

  • Has a very broad spectrum of presentations.
    • Syphilis is often referred to as “The Great Imitator” and congenital syphilis is the same!
    • From asymptomatic to stillbirth.
    • Most neonates will be asymptomatic at birth, but if untreated will manifest symptoms.
Early Congenital Syphilis
  • Present before 2 years of age [Hussain, 2023; Foles, 2024; Fang, 2022; Morrisroe, 2021]
    • Only ~33% of newborns present with symptoms at birth and can be subtle.
    • Most will develop signs by 3 months of age.
  • Rhinitis
    • One of the 1st findings.
    • Usually in 1st week of life
    • Copious, white-ish drainage
    • Teeming with spirochetes! (gross)
  • Rash
    • Occurs 1-2 weeks after rhinitis.
    • 70% of early congenital syphilis will include dermatologic findings.
    • Small macules or papules often on lower extremities, buttock, and back.
    • May be on soles of feet!
    • Progresses to desquamation and crusting.
    • Pemphigus syphiliticus
  • Hepatomegaly
    • Most common
    • May have abnormal LFTs and evidence of Hepatitis.
    • Jaundice
  • Lymphadenopathy
    • Generalized
    • Epitrochlear lymphadenopathy is suggestive of congenital syphilis.
  • CNS findings
    • Seizures
    • Meningitis
    • Hypopituitarism
    • Cranial Nerve Palsies (III, IV, VI, VII)
  • Ocular findings
  • Misc:
Late Congenital Syphilis
  • Presents after 2 years of age [Hussain, 2023; Foles, 2024; Fang, 2022]
    • Asymptomatic patients with congenital syphilis may present after 2 years of age.
    • Most late congenital syphilis can be prevented with appropriate treatment of the mother or by treating the infant within the 1st 3 months of life.
  • Without therapy, progressive inflammation leads to: [Hussain, 2023]
    • Hutchinson’s Triad:
      • Hutchinson teeth (hypoplastic, notched permanent teeth)
      • Interstitial keratitis
      • Sensorineural Hearing Loss (Cranial Nerve VIII)
    • Gumma formations
    • Periostitis
    • Frontal bossing
    • Saddle nose
    • Prominent maxilla
    • Saber shins (anterior bowing)
    • Cognitive deficits
    • Corneal scarring, keratitis, and secondary glaucoma

Congenital Syphilis: Management

Diagnosis of congenital syphilis
  • Darkfield microscopic [Foles, 2024]
    • Examination of nasal discharge if present
    • Can also be used for any material from suspected lesions (ex, skin lesion).
    • Can identify T. pallidum before antibodies against it can be detected.
  • Non-treponemal tests:
    • Venereal Disease Research Laboratory (VDRL) or
    • Rapid Plasma Reagin (RPR)
  • Specific treponemal tests:
    • Fluorescent treponemal antibody absorption (FTA-ABS) and/or
    • Micro hemagglutination test for antibodies to T.Pallidum (MHA-TP) testing
Treatment of congenital syphilis
  • Neonates (birth to 4 weeks of age):
    • In first 7 days of life = Aqueous crystalline penicillin G 50,000 Units/kg IV every 12 hours
    • After 7 days of life = Aqueous crystalline penicillin G 50,000 Units/kg IV every 8 hours
    • Treat for 10-14 days.

  • > 4 weeks of age:
    • Aqueous crystalline penicillin G 50,000 Units/kg IV every 6 hours
    • Treat for 10-14 days.

  • Be aware of Jarisch-Herxheimer reaction
    • Due to an acute inflammatory response from dying T. pallidium with starting treatment.
    • Causes fevers, hypotension, and can be life-threatening.

Moral of the Morsel

  • Maternal Screening Matters… but isn’t perfect! Congenital Syphilis may still occur in patients presenting to your ED!
  • Neonates are congested… but usually not snotty! Consider Congenital Syphilis in the neonate who has lots of “snot”… it may be not, snot… but rather spirochetes! GROSS!!
  • Penicillin is Paramount! It is very effective at killing T. pallidium … of course a lot of spirochetes dying can lead to Jarisch-Herxheimer reaction too.

References

Hussain SA, Leslie SW, Vaidya R. Congenital and Maternal Syphilis. 2024 Apr 21. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. PMID: 30725772.

Foles AI, Eiras Dias M, Figueiredo M, Marçal M. Congenital syphilis: the re-emergence of a forgotten disease. BMJ Case Rep. 2024 Jan 16;17(1):e257694. doi: 10.1136/bcr-2023-257694. PMID: 38233003; PMCID: PMC10806924.

Moseley P, Bamford A, Eisen S, Lyall H, Kingston M, Thorne C, Piñera C, Rabie H, Prendergast AJ, Kadambari S. Resurgence of congenital syphilis: new strategies against an old foe. Lancet Infect Dis. 2024 Jan;24(1):e24-e35. doi: 10.1016/S1473-3099(23)00314-6. Epub 2023 Aug 18. PMID: 37604180.

Fang J, Partridge E, Bautista GM, Sankaran D. Congenital Syphilis Epidemiology, Prevention, and Management in the United States: A 2022 Update. Cureus. 2022 Dec 27;14(12):e33009. doi: 10.7759/cureus.33009. PMID: 36712768; PMCID: PMC9879571.

Morrisroe E, Farzana SF, McKinnon J. Congenital syphilis in a 4-month-old infant with limb weakness. BMJ Case Rep. 2021 Feb 5;14(2):e240532. doi: 10.1136/bcr-2020-240532. PMID: 33547108; PMCID: PMC7871267.

Mannelli L, Perez FA, Parisi MT, Giacani L. A case of congenital syphilis. Emerg Radiol. 2013 Aug;20(4):337-9. doi: 10.1007/s10140-013-1112-1. Epub 2013 Feb 27. PMID: 23443516.

Author

Sean M. Fox
Sean M. Fox
Articles: 586