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
  2. Rapid spread of Treponema pallidum in the fetus, with devastating, often lethal consequences
    1. Associated with a high rate of Stillbirth, Miscarriage and newborn death
    2. Congenital Syphilis in surviving newborns may not be initially evident

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, osteitis)
      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 (widely spaced with central notch)
    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. Disseminated gummata
    2. Neurosyphilis
      1. Deafness (CN 8 palsy)
    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. Prevention

  1. Early Syphilis detection and treatment before pregnancy (or in first trimester)
    1. Congenital Syphilis complications may be completely averted if treated before the 4th month of pregnancy

XI. Resources

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

XII. 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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Related Studies

Ontology: Syphilis, Congenital (C0039131)

Definition (NCI) A life-threatening bacterial infection of the newborn caused by Treponema pallidum. It is transmitted to the infant from a mother with syphilis through the placenta during pregnancy. Signs and symptoms include irritability, fever, failure to thrive, saddle nose, cutaneous rash, and pneumonia.
Definition (MSH) Syphilis acquired in utero and manifested by any of several characteristic tooth (Hutchinson's teeth) or bone malformations and by active mucocutaneous syphilis at birth or shortly thereafter. Ocular and neurologic changes may also occur.
Concepts Disease or Syndrome (T047)
MSH D013590
ICD9 090.9, 090
ICD10 A50 , A50.9
SnomedCT 186845009, 154380005, 187352005, 35742006
English Congenital Syphilis, Syphilis, Congenital, Congenit syphilis, unspecfd, Congenital syphilis, unspecified, [X]Congenit syphilis, unspecfd, [X]Congenital syphilis, unspecified, SYPHILIS CONGEN, CONGEN SYPHILIS, congenital syphilis, congenital syphilis (diagnosis), Congenital Treponema pallidum infection, Unspecified congenital syphilis, Congenital syphilis NOS, Syphilis, Congenital [Disease/Finding], syphilis congenital, Syphilis;congenital, [X]Congenital syphilis, unspecified (disorder), Congenital syphilis NOS (disorder), Congenital syphilis, Congenital syphilis (disorder), congenital; syphilitic, hereditary; syphilitic, syphilis; congenital, Congenital syphilis, NOS
Dutch niet-gespecificeerde congenitale syfilis, congenitale Treponema pallidum-infectie, congenitale syfilis, niet-gespecificeerd, congenitaal; syfilis, hereditair; syfilis, syfilis; congenitaal, Congenitale syfilis, niet gespecificeerd, syfilis, congenitaal, Aangeboren syfilis, Congenitale syfilis, Syfilis, aangeboren
French Syphilis congénitale, non précisée, Infection à Treponema pallidum congénitale, Syphilis congénitale non précisée, Syphilis congénitale
German kongenitale Syphilis, unspezifisch, unspezifische kongenitale Syphilis, kongenitale Treponema pallidum-Infektion, Syphilis connata, nicht naeher bezeichnet, kongenitale Syphilis, Lues congenita, Lues, angeborene, Syphilis connata, Syphilis, angeborene
Italian Infezione congenita da Treponema pallidum, Sifilide congenita, non specificata, Sifilide congenita non specificata, Sifilide congenita
Portuguese Sífilis congénita NE, Infecção congénita por Treponema pallidum, Sífilis congénita, Sífilis Congênita
Spanish Infección congénita por Treponema pallidum, Sífilis congénita no especificada, sífilis congénita, SAI (trastorno), [X]sífilis congénita, no especificada (trastorno), [X]sífilis congénita, no especificada, sífilis congénita, SAI, sífilis congénita (trastorno), sífilis congénita, Sífilis congénita, Sífilis Congénita
Japanese 先天梅毒、詳細不明, 詳細不明の先天梅毒, 先天性トレポネーマパリズム感染症, ｾﾝﾃﾝﾊﾞｲﾄﾞｸ, ｾﾝﾃﾝｾｲﾄﾚﾎﾟﾈｰﾏﾊﾟﾘｽﾞﾑｶﾝｾﾝｼｮｳ, ｾﾝﾃﾝﾊﾞｲﾄﾞｸｼｮｳｻｲﾌﾒｲ, ｼｮｳｻｲﾌﾒｲﾉｾﾝﾃﾝﾊﾞｲﾄﾞｸ, 梅毒-乳児, 先天梅毒, 乳児梅毒, 梅毒-胎児性, 先天性梅毒, 胎児性梅毒, Hutchinson歯, 梅毒-先天性
Swedish Syfilis, medfödd
Czech syfilis kongenitální, Vrozená infekce Treponema pallidum, Vrozená syfilis, Blíže neurčená vrozená syfilis, Vrozená syfilis, blíže neurčená
Finnish Synnynnäinen kuppa
Russian SIFILIS VROZHDENNYI, GUTCHINSONA ZUBY, ГУТЧИНСОНА ЗУБЫ, СИФИЛИС ВРОЖДЕННЫЙ
Korean 상세불명의 선천 매독, 선천 매독
Polish Kiła wrodzona, Zęby Hutchinsona
Hungarian Nem meghatározott veleszületett syphilis, Veleszületett syphilis, nem meghatározott, Veleszületett syphilis, Veleszületett treponema pallidum fertőzés
Norwegian Heredolues, Kongenital syfilis, Lues congenita, Medfødt syfilis
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Clutton's joints (C0239075)

Concepts Disease or Syndrome (T047)
ICD10 A50.51
SnomedCT 19290004
English Clutton joints, syphilis congenital late symptomatic clutton's joints, Clutton's joints (diagnosis), Clutton's joints, Clutton's joint, Clutton's joints (disorder), Clutton (etiology), Clutton (manifestation)
Spanish articulaciones de Clutton (trastorno), articulaciones de Clutton
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Saber shin (C0240920)

Concepts Sign or Symptom (T184)
SnomedCT 47381006
English saber shins (physical finding), saber shins, saber shin, Saber shin (disorder), Saber shin, Saber shin, NOS
Spanish tibia en sable (trastorno), tibia en sable
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Moon's molar teeth (C0266024)

Concepts Congenital Abnormality (T019)
SnomedCT 10557008, 109436001
English mulberry molar (physical finding), mulberry molar, teeth malformed mulberry molar, molar moon's, mulberry molars, Mulberry molar (disorder), Mulberry molar, Mulberry molars, Moon's molars, Moon's molar teeth (disorder), Moon's molar teeth, mulberry molars; molars
Spanish molar en mora (concepto no activo), molar en mora, diente molar de Moon (trastorno), diente molar de Moon
Dutch Mulberry; molaren
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Congenital syphilitic pemphigus (C0275867)

Concepts Disease or Syndrome (T047)
SnomedCT 275373004, 186832005, 59721007
English Cong. syph. pemphigus, congenital syphilitic pemphigus, congenital syphilitic pemphigus (diagnosis), Pemphigus syphiliticus, Congenital syphilitic pemphigus, Congenital syphilitic pemphigus (disorder), pemphigus; syphilitic, syphilis; pemphigus, Syphilitic pemphigus
Dutch pemphigus; luetisch, syfilis; pemphigus
Spanish pénfigo sifilítico congénito (trastorno), pénfigo sifilítico congénito
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Syphilitic saddle nose (C0275873)

Concepts Disease or Syndrome (T047) , Anatomical Abnormality (T190)
ICD10 A50.57
SnomedCT 77782006
English Syphilitic saddle nose, Syphilitic saddle nose (disorder), deformity; saddle nose, syphilitic, saddle nose; due to syphilis, saddle nose; syphilitic, syphilis; saddle nose
Dutch misvorming; zadelneus, luetisch, syfilis; zadelneus, zadelneus; door syfilis, zadelneus; luetisch
Spanish nariz en silla de montar sifilítica (trastorno), nariz en silla de montar sifilítica
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Snuffles in newborn (C0456018)

Concepts Disease or Syndrome (T047)
ICD10 P28.89
SnomedCT 206325007, 271375003
English Neonatal sniffles, Neonatal snuffles, Snuffles in newborn, Snuffles in newborn (finding), newborn; snuffles, snuffles; newborn
Dutch pasgeborene; verstopte neus, verstopte neus; pasgeborene
Spanish coriza neonatal, resuello neonatal (hallazgo), resuello neonatal
Derived from the NIH UMLS (Unified Medical Language System)

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