Transcript Case presentation - The Department of Pediatrics of

Joint OB / Pediatrics M&M
conference
• PERINATAL CASE PRESENTATION
AND DISCUSSION OF
SEROLOGYCALLY POSITIVE MOTHER
and INFANT FOR SYPHILIS
Christian Castillo, MD
BK Rajegowda, MD
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
Presentation of cases Mother’s profiles
Case #1 MR#2310021
Case #2 MR#2310056
Case #3 MR#2310550
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 1:4
2/23/09 RPR 1:32 first visit
12/17/08 RPR 1:8 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 1:8 No Tx
3/23/09 RPR 1:8 no Tx
Visit 4/7/09 refers to past Tx
but not documentation
Day of
Delivery
3/28/09
4/4/09
Follow up
Serology after
Birth
No follow up serology.
Post Natal visit 4/13/09
4/6/09 after delivery RPR 1:4
4/6/09 Pen G 2.4 mill
4/19/09
4/19/09 RPR 1:16
Mother tx after delivery
Patient’s 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 @ 1 min and 9 @ 5 min
BWt: 3495 gms; L: 50.5 cms; HC: 34.5 cms; CC: 35 cms; Ag: 33 cms;
SROM at 18:30hrs the day PTD, 13hr PTD; AF: clear
Time of birth: 07:23hrs 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 1:2 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 Patient’s RPR no reactive TPPA reactive IgG ab reactive
Patient’s profile
Case # 2
Maternal Late registrant, PNC X 5 LH RPR 1:32
no follow up titers
• Delivery 4/4/09
• FT AGA, NSVD at 38.1 by LMP to 24 y/o G6P3024
APGAR 9@ 1 min and 9 @ 5 min
B Wt: 3535 g, Length: 52.5 cm, HC: 35 cm, CC: 34 cm, AC: 35.5 cm
ROM: 6 . AF: clear at the time of birth 10.07am
normal VS and PE
• 4/4/09 Cord RPR 1:16 TPPA reactive
• 4/5/09 Patient Plasma RPR 1:16 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 1:8 TPPA reactive
• 4/10/09 Pen G benz
• 4/10/09 Discharge
Patient’s profile
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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, G3P0020
APGAR: 9 @ 1 min and 9 @ 5 min
B 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 1:4 TPPA reactive
4/19/09 and 4/21/09 Patient Plasma RPR 1:4 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
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.
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
P&S 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
CDC Congenital Syphilis
Reported cases and rates in infants < 1 year
2003-2007
State/Area*
2003
2004
Cases
2005
Georgia
Hawaii
Idaho
Illinois
Louisiana
Maine
Maryland
Massachusetts
Michigan
Nevada
New Jersey
New Mexico
NEW YORK
North Carolina
Oklahoma
Oregon
Pennsylvania
Texas
Washington
West Virginia
Wisconsin
Wyoming
U.S. TOTAL
11
2
4
20
6
0
9
0
38
0
21
6
42
20
1
0
2
77
0
0
0
0
432
6
0
3
26
19
0
10
0
23
1
13
3
22
9
2
0
0
65
0
0
1
0
375
1
0
0
23
13
0
16
0
17
1
16
6
10
11
1
0
1
67
0
0
2
0
339
2006
2007
9
0
0
15
16
0
19
0
13
16
15
7
24
7
2
0
4
79
0
0
0
0
373
9
0
0
10
36
0
23
0
14
7
11
6
16
7
3
2
8
99
2
1
1
0
430
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
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.
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.
Evaluation of Newborn with Congenital
Syphilis
Mother’s 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 > 4 folds and/or
infant’s titer is 4 fold greater than the mother’s titers
complete workup is warrant.
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 < 1 month prior to delivery
Syphilis treated before pregnancy but with insufficient
serologic f/u to assess response
Evaluation of Newborn with Congenital
Syphilis -work upPhysical 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
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.
Congenital Syphilis
Hydrops fetalis
Nasal
discharge
Petechial rash
Necrotizing
funisitis
within the matrix of
the umbilical cord
Hepatomegaly
Rash
Ostitis ,
Metaphysitis,
Periostitis
Wimberger
sign
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
Radiografic Abnormalities
A more specific finding is localized
bony destruction of the medial portion
of the proximal tibial metaphysis
(Wimberger’s sign). Other findings
include metaphyseal serration
(“sawtooth metaphyses”), and
diaphyseal involvement with
periosteal reaction.
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.
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 mother’s 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 Regimens
Aqueous crystalline penicillin G 100,000–150,000 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
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 <4 weeks before delivery.
Recommended Evaluation
• CSF analysis for VDRL, cell count, and protein -CBC and PLT Long –bone RX
Recommended Regimens
Aqueous crystalline penicillin G 100,000–150,000 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
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 >4 weeks before delivery;
and
•mother has no evidence of reinfection or relapse.
Recommended Evaluation
No evaluation is required.
Recommended Regimen
Benzathine penicillin G 50,000 units/kg/dose IM in a single dose
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
-Mother’s treatment was adequate before pregnancy, and
-mother’s nontreponemal serologic titer remained low and stable before
and during pregnancy and at delivery (VDRL <1:2; RPR <1:4).
• 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.
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.
• Thank you !!