Joint OB / Pediatrics M

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Joint OB / Pediatrics MM conference

• PERINATAL CASE PRESENTATION AND DISCUSSION OF
  SEROLOGYCALLY POSITIVE MOTHER and INFANT FOR
  SYPHILIS

Christian Castillo, MD BK Rajegowda, MD
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Congenital Syphilis

• Syphilis is a Sexually transmitted disease
• Congenital Syphilis is a consequence of untreated
  or Inadequately treated maternal syphilis
• Rare but still occurs. A recent increase
• in cases is reported
• Prevention, early diagnosis and treatment will
  prevent fetal and neonatal infections

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Presentation of cases Mothers profiles
Case 1 MR2310021 Case 2 MR2310056 Case 3 MR2310550
Age 19 24 19
Race Hispanic Black African American Caucasian
Parity G3P0020 G6P3024 G3P0020
PNC Neighborhood Health Center ??? First time in LH LH X 5 Late registrant at 34wk LH X 10 late registrant at 19 wk
Time / Date serology RPR by Hx reactive and treated 2yrs ago at the health department.No documentation RPR 3/27/09 14 2/23/09 RPR 132 first visit 12/17/08 RPR 18 1st visit
Treatment 3/28/09 Penicillin B 2.4 IM 4/13/09 PNC 2 after Delivery at the clinic 3/6/09 Pen G 2.4 mill second V 3/20/09 pen G 2.4 mill 1/26/09 Documented only prescription given for Pen B X 3
Follow up serology tx 4/1/09Patient DC AMA No follow up titers before delivery 3/9/09 RPR 18 No Tx 3/23/09 RPR 18 no Tx Visit 4/7/09 refers to past Tx but not documentation
Day of Delivery 3/28/09 4/4/09 4/19/09
Follow up Serology after Birth No follow up serology. Post Natal visit 4/13/09 4/6/09 after delivery RPR 14 4/6/09 Pen G 2.4 mill 4/19/09 RPR 116 Mother tx after delivery
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Patients profile
Case 1 Maternal tx undocumented, unknown
PNC Delivery 3/28/09 FTAGA female born via C
section at 40.3w by LMP Apgar 9 _at_ 1 min and 9 _at_
5 min BWt 3495 gms L 50.5 cms HC 34.5 cms
CC 35 cms Ag 33 cmsSROM at 1830hrs the day
PTD, 13hr PTD AF clearTime of birth 0723hrs
Normal VS and PE In view of unknown Labs and
treatment prior to delivery, normal PE we decided
to work up and treat this baby as unlikely
syphilis Cord RPR 3/28/09 12 TPPA reactive
CBC 30.9/19.3/59/212 N73 Band 3 L 15 Long bone
X ray , WNL CSF studies RBC 19519 WBC 5 Seg 70
Lymp 25 Mono 3 Eos 2 Glucose 46 protein 141 VDRL
CSF no reactive 4/1/09 Tx Pen Benz 175000 Units
IM 4/1/09 Discharge patient 5/7/09 Serum
Patients RPR no reactive TPPA reactive IgG ab
reactive
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Patients profile

• Case 2
• Maternal Late registrant, PNC X 5 LH RPR 132
• no follow up titers
• Delivery 4/4/09
• FT AGA, NSVD at 38.1 by LMP to 24 y/o
  G6P3024APGAR 9_at_ 1 min and 9 _at_ 5 minB Wt 3535
  g, Length 52.5 cm, HC 35 cm, CC 34 cm, AC
  35.5 cmROM 6 . AF clear at the time of birth
  10.07amnormal VS and PE
• 4/4/09 Cord RPR 116 TPPA reactive
• 4/5/09 Patient Plasma RPR 116 TPPA reactive
• 4/6/09 CSF studies RBC 475 WBC 4 Glucose 38
  protein 132 VDRL CSF no reactive
• 4/7/09 Long bones X- R WNL
• 4/7/09 , 4/8/09 / 4/9/09 Tx Pen Procaine until
  VDRL CSF no reactive
• 4/9/09 RPR 18 TPPA reactive
• 4/10/09 Pen G benz
• 4/10/09 Discharge

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Patients profile

• Case 3
• Maternal Late registrant, PNC X 10 LH incomplete
  Treatment
• Delivery 4/19/09
• FTAGA, NSVD at 39.6 weeks by LMP to 19 y/o
  Caucasian, G3P0020APGAR 9 _at_ 1 min and 9 _at_ 5
  minB Wt 3085 g, Length 49 cm, HC 34. cm, CC
  31 cm, AC 33.5 cm ROM 12 hrs ptd. AF clear
• Normal PE
• 4/19/09 Cord RPR 14 TPPA reactive
• 4/19/09 and 4/21/09 Patient Plasma RPR 14 TPPA
  reactive
• 4/21/09 CSF RPR NR Cell count RBC 1 WBC 4 clear.
  Glucose 44 protein 84
• 4/21/09 4/22/09 4/23/09 Pen Procaine 50,000
  Units/Kg
• 4/21/09 Long bones X ray . WNL
• 4/24/09 Pen G benz 50, 000 units / Kg
• 4/24/09 Discharge
• 5/6/09 RPR no Reactive IgG reactive

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Congenital Syphilis

• The incidence of congenital syphilis corresponds
  to the incidence of disease in women.

Incidence increased dramatically during late
1980 and early 1990 but subsequently decreases.
In almost three quarter of cases the mother was
not treated, or was inadequately treated.
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Congenital Syphilis
Congenital Syphilis  United States After 14
years of decline in the United States, the rate
of congenital syphilis increased 15.4 between
2006 and 2007 (from 9.1 to 10.5 cases per 100,000
live births). In 2007, 430 cases were reported,
an increase from 373 in 2006. This increase in
the rate of congenital syphilis may relate to the
increase in the rate of PS syphilis among women
that has occurred in recent years . Congenital
Syphilis by State In 2007, 29 states had rates of
congenital syphilis that exceeded the 2010 target
of one case per 100,000 live births . NYS
reported 6.4 /100000 in 2007
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CDC Congenital Syphilis Reported cases and rates
in infants lt 1 year 2003-2007
State/Area Cases Cases Cases Cases Cases
State/Area 2003 2004 2005 2006 2007
Georgia 11 6 1 9 9
Hawaii 2 0 0 0 0
Idaho 4 3 0 0 0
Illinois 20 26 23 15 10
Louisiana 6 19 13 16 36
Maine 0 0 0 0 0
Maryland 9 10 16 19 23
Massachusetts 0 0 0 0 0
Michigan 38 23 17 13 14
Nevada 0 1 1 16 7
New Jersey 21 13 16 15 11
New Mexico 6 3 6 7 6
NEW YORK 42 22 10 24 16
North Carolina 20 9 11 7 7
Oklahoma 1 2 1 2 3
Oregon 0 0 0 0 2
Pennsylvania 2 0 1 4 8
Texas 77 65 67 79 99
Washington 0 0 0 0 2
West Virginia 0 0 0 0 1
Wisconsin 0 1 2 0 1
Wyoming 0 0 0 0 0
U.S. TOTAL 432 375 339 373 430
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Congenital Syphilis Clinical Presentation

• Congenital syphilis lack a primary stage
• because it is disseminated through blood
• Fetal infections can occur at any time during
  pregnancy
• Hepatomegaly is present in almost 100
• Necrotizing funisitis within the matrix of the
  umbilical cord is consider highly indicative
• 60 of patients are asymptomatic

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Maternal Syphilis Dx and treatment
Test During Pregnancy All women should be
screened for syphilis with a non Treponemal test
RPR / VRDL early in pregnancy and preferably
again at delivery . In high risk areas testing
at the beginning of 3rd Trimester is also
recommended. All Positive tests should be
confirmed with a Treponemal test FTS-ABS
/TPPA. For women treated during pregnancy FU
serology testing is necessary to assess efficacy
of therapy. Treatment with penicillin is the
gold standard.
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Maternal Syphilis Dx and treatment

• A single dose of Benzathine Penicillin therapy
  for early disease is only appropriate when is
  possible to document that there was a non
  reactive Syphilis test within the last Year.
• Some Give a second dose of Benzathine Penicillin
  1 week after the first to improve the likelihood
  of a serology response in early disease.
• In all other cases the disease should be consider
  Latent syphilis of unknown duration for which 3
  doses of Benzathine penicillin at weekly
  intervals are recommended.
• Follow up titers at 1,3,6,12 and 24 months
  decreases fourfold by 6 months and becomes
  negative by 12-24 months. Failure to decrease
  titers is likely to be failure to treat or
  reinfection.

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Evaluation of Newborn with Congenital Syphilis

• Mothers serological status for syphilis
• Blood cord testing is inadequate for screening
  (could be non-reactive even when the mother is )
• Infants born from seropositive mothers require a
  careful examination and a quantitative
  non-treponemal test (same test should be
  performed to the mother)
• If maternal titers have increased to gt 4 folds
  and/or infants titer is 4 fold greater than the
  mothers titers complete workup is warrant.

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Evaluation of Newborn with Congenital Syphilis

• Untreated, inadequately treated, or treatment not
  documented
• Treated with a non-penicillin regimen
  (i.e.,erythromycin)
• Appropriately treated with PNC, but without the
  expected decrease in treponemal titers
• Syphilis treated lt 1 month prior to delivery
• Syphilis treated before pregnancy but with
  insufficient serologic f/u to assess response

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Evaluation of Newborn with Congenital Syphilis
-work up-

• Physical Examination
• Quantitative non-treponemal serologic test of
  serum from the infant for syphilis (not from cord
  blood)
• VDRL and cell count from CSF
• Long bone X-rays (unless Dx established
  otherwise)
• Complete blood cell and platelet count
• Other tests include
• Chest X-ray
• LFT
• Pathological examination of placenta or umbilical
  cord using specific fluorescent antitreponemal
  antibody staining
• Vision and hearing test

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Evaluation of Newborn with Congenital Syphilis

• Transplacental transmission of nontreponemal and
  treponemal antibodies to the fetus can occur in a
  mother who has been treated appropriately for
  syphilis during pregnancy, resulting in
  uninfected newborns, usually reverting by 4 to 6
  months of age, whereas FTA-ABS or TP-PA test
  result from passively acquired Ab and it may not
  become negative for 1 year or longer.

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Congenital Syphilis
Hydrops fetalis
Nasal discharge
Petechial rash
Necrotizing funisitis within the matrix of the
umbilical cord
Hepatomegaly
Rash
Ostitis , Metaphysitis, Periostitis Wimberger
sign
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Decreased mineralization of the metaphyses of
long bones of the upper extremities
bilateral lytic lesions of the talus, calcaneous,
and proximal tibia (Wimberger sign) medially
A more specific finding is localized bony
destruction of the medial portion of the proximal
tibial metaphysis (Wimbergers sign). Other
findings include metaphyseal serration (sawtooth
metaphyses), and diaphyseal involvement with
periosteal reaction.
Radiografic Abnormalities
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Dermatology finding Congenital Syphilis
Dermatological findings are quite variable,
although palmar/plantar, perioral, and anogenital
regions are classically described as being
involved.  The images to the left demonstrate
findings at birth in an affected infant, with a
desquamating eruption that was widespread over
the entire body.  These lesions are extremely
infectious.  Because of the variable lesions and
clinical symptoms seen with CS, it has frequently
been termed "the great imitator", and it is
important to consider alternative diagnoses or
vesiculobullous diseases that involve the palms
and soles.
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CDC guideline 2006 Congenital Syphilis
Scenario 1. Infants with proven or highly
probable disease and -an abnormal physical
examination that is consistent with congenital
syphilis, -a serum quantitative nontreponemal
serologic titer that is fourfold higher than the
mothers titer, or -a positive dark field or
fluorescent antibody test of body fluid(s).
Recommended Evaluation CSF analysis for VDRL,
cell count, and protein CBC and PLT Other
tests as clinically indicated (e.g., long-bone
radiographs, chest radiograph, liver-function
tests, cranial ultrasound, ophthalmologic
examination, and auditory brainstem response)
Recommended RegimensAqueous crystalline
penicillin G 100,000150,000 units/kg/day,
administered as 50,000 units/kg/dose IV every 12
hours during the first 7 days of life and every 8
hours thereafter for a total of 10 days    OR
Procaine penicillin G 50,000 units/kg/dose IM
in a single daily dose for 10 days
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CDC guideline 2006 Congenital Syphilis

• Scenario 2. Infants who have a normal physical
  examination and a serum quantitive nontreponemal
  serologic titer the same or less than fourfold
  the maternal titer and the
• -mother was not treated, inadequately treated, or
  has no documentation of having received
  treatment
• -mother was treated with erythromycin or other
  nonpenicillin regimen or
• -mother received treatment lt4 weeks before
  delivery.
• Recommended Evaluation
• CSF analysis for VDRL, cell count, and protein
  -CBC and PLT -Long bone RX
• Recommended RegimensAqueous crystalline
  penicillin G 100,000150,000 units/kg/day,
  administered as 50,000 units/kg/dose IV every 12
  hours during the first 7 days of life and every 8
  hours thereafter for a total of 10 days    OR
  Procaine penicillin G 50,000 units/kg/dose IM in
  a single daily dose for 10 days    OR
  Benzathine penicillin G 50,000 units/kg/dose IM
  in a single dose
• Some specialists prefer the 10 days of parenteral
  therapy if the mother has untreated early
  syphilis at delivery

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CDC guideline 2006 Congenital Syphilis

• Scenario 3. Infants who have a normal physical
  examination and a serum quantitative
  nontreponemal serologic titer the same or less
  than fourfold the maternal titer and the
• mother was treated during pregnancy, treatment
  was appropriate for the stage of infection, and
  treatment was administered gt4 weeks before
  delivery and
• mother has no evidence of reinfection or relapse.
• Recommended Evaluation No evaluation is
  required.
• Recommended RegimenBenzathine penicillin G
  50,000 units/kg/dose IM in a single dose

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CDC guideline 2006 Congenital Syphilis

• Scenario 4. Infants who have a normal physical
  examination and a serum quantitative
  nontreponemal serologic titer the same or less
  than fourfold the maternal titer and the
• -Mothers treatment was adequate before
  pregnancy, and
• -mothers nontreponemal serologic titer remained
  low and stable before and during pregnancy and at
  delivery (VDRL lt12 RPR lt14).
• Recommended Evaluation No evaluation is
  required.
• Recommended Regimen No treatment is required
  however, some specialists would treat with
  benzathine penicillin G 50,000 units/kg as a
  single IM injection, particularly if follow-up is
  uncertain.

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Congenital Syphilis

• Conclusions
• The incidence of congenital syphilis corresponds
  to the incidence of disease in women.
• All pregnant women should be tested 1st trimester
  and in the beginning of 3rd Trimester and at
  delivery.
• All positive test should be confirmed with a
  Treponemal Test , treat and follow up titers as
  per protocol.
• Documentation is an important aspect in the
  evaluation of treatment.

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• Thank you !!