Congenital Syphilis December 10, 2010 Renata Dennis, RN, MPH Southeast AIDS Training and Education Center (SEATEC) Emory University School of Medicine.

Congenital Syphilis

December 10, 2010

Renata Dennis, RN, MPHSoutheast AIDS Training and Education Center (SEATEC)Emory UniversitySchool of MedicineAtlanta, GA 30322

Disclosure•“I have no real or perceived vested interests

that relate to this presentation nor do I have any relationships with pharmaceutical companies, biomedical device manufacturers, and/or other corporations whose products or services are related to pertinent therapeutic areas.”

Renata Dennis, RN, MPH, 10 December 2010

Objective

By the end of this session, participants will be able to:

•Describe the clinical manifestations of congenital syphilis

•Discuss maternal and infant testing for congenital syphilis

•Describe a prevention plan for congenital syphilis

How common are STDS in pregnant women in the United States?

Estimated Number of

STDs Pregnant WomenBacterial Vaginosis 1, 080,000Herpes Simplex virus 2 880,000Trichomoniasis 124,000Chlamydia 100,000Gonorrhea 13,200Hepatitis B 16,000HIV 6,400Syphilis <1,000

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CDC Fact Sheet, updated December 2007

*TORCH Infections in Pregnancy

T-ToxoplasmosisO-Other Infections (HIV, Hepatitis B, Syphilis,

etc.)R-RubellaC-CytomegalovirusH-Herpes Simplex

*Also known as STORCH or TORCHeS (to account for the importance of Syphilis), plus other acronyms to emphasize a variety of important infections.

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Tolan, R., Infectious Diseases in Clinical Practice, 01/2008

Primary and secondary syphilis — Rates among women by state: United States and outlying areas, 2008

Note: The total rate of P&S syphilis among women in the United States and outlying areas (Guam, Puerto Rico, and Virgin Islands) was 1.5 per 100,000 female population.

Rate per 100,000population

Guam 1.2

Puerto Rico 1.4

V irgin Is. 0.0

<=0.2

0.21-4.0

>4.0

(n= 17)

(n= 33)

(n= 4)

VT 0.0NH 0.0MA 0.2RI 0.0CT 0.0NJ 0.5DE 1.3MD 2.7DC 2.3

7.2

0.0

1.8 5.6

0.6 0.1

2.1

2.0

1.1

0.1

0.6 0.5

0.2

0.40.6

13.9

0.0

1.2

0.2

4.4

1.0

0.2

0.31.1

0.6

0.5

0.9

0.0

1.1

1.4

0.1

0.3

0.5

0.3

3.8

3.8

0.10.6

0.2

0.4

0.5

0.8

Congenital Syphilis: Epidemiology and Pathogenesis• Perinatal transmission of Treponema pallidum at any

stage of pregnancy or during delivery from an infected woman to her offspring

• Drug use during pregnancy increases risk of maternal and congenital syphilis

• Syphilis chancres make it easier to transmit and acquire HIV infection sexually, and

• Rate of congenital syphilis 50 times greater among infants born to HIV-infected mothers

AETC National Resource Center, Treating Opportunistic Infection Among HIV-Infected Children, 12/2004STD Fact Sheet, www.cdc.gov/std/Syphilis/STDFact-Syphilis.htm, downloaded 12/2010

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Congenital Syphilis: Epidemiology and Pathogenesis, cont’d.•The spirochete crosses the placenta and infects

the fetus; may also occur from contact with an infectious lesion canal during delivery

• In utero sonographic findings include: hydrops, thickened placenta, hepatomegaly, ascites

•Any woman delivering stillbirth after 20 weeks gestation should be evaluated for syphilis

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CDC Syphilis Surveillance Report, 2006; S. Philip, Congenital Syphilis, 9/2002

Case(s) of Congenital Syphilis ????Henry VIII, King of England,

1491-1547

• Much controversy among historians

• Wives had many miscarriages and stillbirths

• The king himself had evidence of a skin disease at 22, series of headaches at 37, open leg ulcers that did not heal at 44, deformity (?gummata) on right side of nose at 45, and then, there was that temper later in life

• NO WAY TO KNOW without testing-- syphilis mimics other conditions

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Farella, C. What if....? Pondering Clinical Cliffhangers, Nursing Spectrum, 2002

Congenital Syphilis:Clinical Symptoms and ManifestationsThree major classifications:

•Fetal effects•Early effects•Late effects

Damage to fetus/infant depends on the stage of development at which the infection has taken place and time elapsed before treatment.

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A. Maranich, TORCH Infections, downloaded 12/2010S. Philip, Congenital Syphilis, 9/2002

Fetal effects(prenatal/perinatal period)

•Miscarriage

•Stillbirth

•Neonatal death

Intrauterine and/or perinatal death in 25-40% of cases

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AETC National Resource Center, Treating Opportunistic Infection Among HIV Infected Children, 12/2004;

A. Aaranich, Torch Infections, downloaded 12/2010

• Hepatosplenomegaly- diffuse inflammation, scarring, jaundice

• Generalized lymphadenopathy –epitrochclear nodes

• Coombs – hemolytic anemia, thromobocytopenia, leukopenia, leukocytosis

• Hydrops fetalis

• Mucocutaneous: rhinitis (highly

infectious), “snuffles”, mucous patches

• Maculopapular rash

• Desquamation

• Pemphigus syphiliticus (vesicular bullous eruptions of palms and soles)

• Petechial lesions

• Bony lesions, osteochondritis, periostitis, pseudoparalysis

• Syphilitc leptomeningitis

• Chorioretinitis, salt and pepper fundus, glaucoma

• Pancreatitis

Early manifestations are varied, with multi-system involvement

Early effects- in up to 60% of infants(usually evident within first 5 weeks, can take up to 2 years)

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S.Philip, Congenital Syphilis, 9/2002A.Maranich, TORCH Infections, downloaded 12/2010

AETC National Resource Center, Treating Opportunistic Infection Among HIV-Children, 1/2004

Papulosquamos Plaques

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S. Philip, Congenital Syphilis, 9/2002

Broken vesicles, desquamation

Condylomata around anusInfiltration, desquamation and rhinitis

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S. Philip, Congenital Syphilis, 9/2002

Rhinitis (snuffles), mucous patches, damage to palate(late) Bullae and vesicular rash on soles

Eroded papular lesions

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S.Philip, Congenital Syphilis, 9/2002

Osteochondritis of distal radius and ulna

Osteochondritis of femur and tibia metaphysis

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S. Philip, Congenital Syphilis, 9/2002

Remember:

•Infants may be asymptomatic at birth

•And without early antibiotic treatment, children may develop:▫Brain damage▫Blindness▫Hearing impairment▫Bone and tooth abnormalities

CDC STD Treatment Guidelines 2006

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• Interstitial keratitis (inflammatory)

• Nerve deafness

• Clutton’s Joints (synositis, restricted movement)

• Hutchinson’s triad (teeth, intersitial keratitis, 8th nerve deafness)

• Mulberry molars

• Flaring scapulas

• Hydrocephalus

• Mental retardation• • Frontal bossing, saddle

nose, protruding mandible

• High arched palate

• Perioral fissures

• Higouemenaki’s sign(periosteal rotation of clavicle, sternocleidomastoid)

• Saber shins

• Rhagades (linear scars that become fissured or ulcerated)

Results primarily from chronic inflammation of bone, teeth,and CNS.

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S. Philip, Congenital Syphilis, 9/2002

Late effects (manifestations after 2 years of age)

Hutchinson’s teeth – peg shaped upper incisors

Frontal Bossing

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S. Philip, Congenital Syphilis, 9/2002

Saber shins: Anterior bowing of tibias Gumma: Thin atrophic scar

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S. Philip, Congenital Syphilis, 9/2002

Diagnosis in newborns, (difficult because of maternal antibodies)

•Confirmed if organism identified in skin lesions•Presumptive, if present: physical exam findings CSF findings (positive VDRL) Osteitis on long bone x-rays Funisitis (“barber shop pole” umbilical cord) Positive IgM antibody (test is hard to get done)•Regardless of physical findings, if mother was

un-or inadequately treated for syphilis

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AETC National Resource Center, Treating Opportunistic Infection Among HIV-Infected Children, 12/2004 A.Maranich, TORCH Infections, downloaded 12/2010

CDC 2006 STD Treatment Guidelines, downloaded 12/2010

Diagnosis in newborns, cont’d.

•Use combination of physical, radiologic, serologic, and direct microscopic (darkfield microscopy, flourescent antibody test of a sample)results, as standard serologic tests detect only IgG

•All infants born to mothers with reactive nontreponemal and treponemal test should be evaluated with a quantitative nontreponemal test, e.g., slide test, rapid plasma reagin (RPR)

AETC National Resource Center, Treating Opportunistic Infection Among HIV-Infected Children, 12/2004

CDC 2006 Sexually Transmitted Diseases Treatment Guidelines, downloaded 12/2010

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•If infant’s titer higher than mother’s congenital infection

•If decreasing titer in infant passive transfer of antibodies, should disappear by 3-4 months of age.

•Persistently reactive VDRL, with rising titer active infection

Remember:•A sustained 4-fold decrease in titer of nontreponemal test after treatment indicates adequate therapy

•A 4-fold titer increase after treatment suggests re-infection or relapse.

Diagnosis in newborns, cont’d.

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S. Philip, Congenital Syphilis, 9/2002

Serologic tests are the principal means for diagnosis:

1) Nontreponemal test: VDRL, RPR

2) Treponemal tests: TPI, FTA-ABS, MHA-TP

Syphilis Diagnosis: A Review

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S. Philip, Congenital Syphilis, 9/2002

Testing for Syphilisswitching tests can cause confusing (VDRL versus RPR)—use the same test to compare and track progress

Nontreponemal:VDRL, RPR

Treponemal:TPI, FTA-Abs, MHA-TP

• Quantitative results correlate with disease activity, therefore, this is a helpful screening test; these tests are sensitive, but not specific

• Titers rise when disease is active, fall when treatment is adequate

• In congenital infection, these tests become non-reactive within a few months of adequate treatment

• Used as a confirmatory test

• Treponemal antibody titers become positive soon after initial infection and usually remain positive for life, even with adequate therapy

• Antibody titers do not correlate with disease activity, and are not quantified

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S. Philip, Congenital Syphilis, 9/2002

Treatment for Congenital Syphilis(Dependent upon clinical scenario)

• Aqueous crystalline penicillin G: 100,000-150,000 units/kg/day, administered as 50 units/kg/dose IV every 12 hours during the first 7 days of life and every 8 hours thereafter for 10 days

OR• Procaine penicillin G: 50,000 units/kg/dose IM in a single daily dose

for 10 daysOR• Benzathine penicillin G: 50,000 units/kg/dose IM in a single dose

• Dosage varies for older infants and children

• Other drugs are an option if there is a penicillin allergy (although desensitization may be possible)

If >1 day of therapy is missed, the entire course should be restarted

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CDC 2006 STD Treatment Guidelines

Should have careful follow-up examination at , 2, 4, 6, and 12 months of age.

Serologic non-treponemal tests: 3, 6, 12 months, and end of treatment (or until non-reactive)

Non-treponemal Ab titers decline by 3 months of age, and should be non-reactive by 6 months, if infant was not infected. (Transplacentally acquired antibodies.)

If persistent, stable titers, consider retreatment.

Congenital neurosyphilis- CSF exam at 6 month intervals until normal.

Close follow-up of infants:

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S. Philip, Congenital Syphilis, 9/2002

Some questions for you:

•When does your agency test pregnant women for syphilis? Is one time per pregnancy enough?

•When do you treat pregnant women? Does this change if she has HIV?

•Do you ask about a history of syphilis on intake? Remember: some people don’t realize you can be re-infected.

A Final Word (from the CDC):

“No infant or mother should leave the hospital unless the maternal serologic status has been documented at least once during pregnancy, and at delivery in communities and populations in which the risk for congenital syphilis is high.”

Added editorial note: The same goes for HIV.

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CDC 2006 STD Treatment Guidelines

CDC MMWR STD Guidelines, 2006*

http://www.cdc.gov/std/treatment/2006/congenital-syphilis.htm

http://www.cdc.gov/std/treatment/2006/n5511.pdf

*New guidelines are expected any day!

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