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CongenitalSyphilis
N.Frewan, PL2
Neonatology Divisio July 2008
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%ntro"ution
.u$a!le ST caused !y theT$eponema pallidum o$)anism
199/ .omplete )enetic seuence
o #. palli"u$ was pu!lishedwhich helped unde$standin) thepatho)enesis o syphilis
Belon)s to 'Spi$ochaetaceae
amily*The )enus name, #repone$a, is
de$i(ed $om the #$ee2 te$m o$tu$nin) th$ead'
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#repone$a Palli"u$
Thin
3otile
4t$emely astidiousSu$(i(e only !$ie6y outside
host
7ot culti(ated successully ona$ticial media
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#rans$ission
Direct sexual contact with ulcerativelesions of skin or mucous membranes
Trans placental:
- Typically during second half of pregnancy- As early as 6 weeks of gestation- Pregnant with primary or secondary
syphilis are more likely to transmit the
disease than those with latent (notclinically apparent disease
Cannot be spread through contact with toilet seats, doorknobs, swimming pools, hot tubs,bathtubs, shared clothing, or eating utensils
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Congenital syphilis
Se(e$e, disa!lin), and oten lie-
th$eatenin) inection seen ininants
8!out hal o all inected etusesdie sho$tly !eo$e o$ ate$ !i$th
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%ni"ene&S
Despite the fact of being curable if
caught early! rising rates among
pregnant " in the #$ have recently %the number of infants born with
congenital syphilis
&')*&''+: overall incidence % ,)-
( $ex*Drug traffic
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%ni"ene&S
&'': &./- of reported cases of 0$occurred because the mother receivedno1inade2uate penicillin tx before orduring pregnancy
According to the 0D0:
- 3+- of births are stillborn- 3+*,+- of the survivors will be infected- 4 &5- of these will subse2uently die
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Reported cases for infants < 1 year of age andrates of 1ry & 2ry syphilis among women:
United States, 1997–2!
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Rates for infants < 1 year of age: US, 1997–2! and the "ealthy #eople 21 target as
per S$% sr'eillance
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ul-:00;
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Pathophysiology CS
T$ans placental t$ansmission
T$ansmission $ate< ;0 - 100=
>ith ea$ly onset disease,maniestations $esult $omt$ans placental spi$ochetemiaand a$e analo)ous to seconda$y
sta)e o acui$ed syphilis.S does not ha(e a p$ima$y
sta)e
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Clinial 'ani(estations
"nt$a-ute$ine -?lacenta
-%etus
?ost-natal - 4a$ly
- ate
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%ntra)uterine* Plaenta
The placenta is typically la$)e andedematous
.ha$acte$istic placental ndin)s include
- Hyd$ops placentalis- .h$onic (illitis
- ?e$i(illous !$ous p$olie$ation
-7o$mo!lastemia
- 7ec$oti@in) unisitis
- 8cute cho$ioamnionitis
- ?lasma cell deciduitis
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%ntra)uterine* Fetus
epends on sta)e o de(elopmentat time o inection & du$ation ount$eated inection
"nitially cha$acte$i@ed !y placentalin(ol(ement and hepaticdysunction +eA)A, a!no$mal %T,ollowed !y amniotic 6uid
inection, hematolo)ica!no$malities, ascites, and hyd$ops
Still!i$th 7eonatal death
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%ntra)uterine* Fetus
C:D wee2s )estation ;; = oetuses ha(e eithe$ con)enitalsyphilis o$ TA?allidum detected in
amniotic 6uid
"nt$aute$ine death :5 = o afected
?e$inatal mo$tality :5-E0 =, iunt$eated
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Post)Natal
8mon) su$(i(o$s, maniestations !eendi(ided into
4a$ly sta)e F %i$st : yea$s
ate sta)e F 8te$ :yea$s
"n6ammato$y chan)es do not occu$ inthe etus until ate$ $st t$imeste$ Go$)ano)enesis is unafected
7e(e$theless, all o$)an systems may !ein(ol(ed
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+arly CS) sy$pto$ati
ccu$s !etween 0 - : yea$s " asymptomatic
- "dentied on $outine p$enatalsc$eenin)
- " not identied and t$eated,these new!o$ns de(elop poo$
eedin) and $hino$$heaI 4a$liest si)ns o .S may !e poo$
eedin) and snuJes +iAeA,syphilitic $hinitis
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Sy$pto$ati +arly CS
" Symptomatic
Ka$ia!le8ppea$ within 1st 5 wee2s o lieStill!o$n ?$ematu$e
%ailu$e to )ain wei)ht o$ %TT%e(e$ "$$ita!ilitySe(e$e con)enital pneumonia
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Sy$pto$ati +arly CS
3ost st$i2in) lesions afect themucocutaneous tissues and !ones
- 3ucous patches
- Lhinitis FsnuJe
- .ondylomatous lesions
I I hi)hly cha$acte$istic eatu$es omucous mem!$ane in(ol(ement in.S
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Sy$pto$ati +arly CS
SnuJes G %ollowed uic2ly !ydifuse maculopapula$desuamati(e $ash that in(ol(esetensi(e slou)hin) o theepithelium, on the palms & solesand a$ound the mouth & anus
>hen ch$onic G 'Saddle 7ose* esions & nasal 6uid hi)hly
inectious
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Sy$pto$ati +arly CS
Bullous s2in disease 2nown as'pemphi)us syphiliticus*
M 4a$ly $ash -- small !liste$s onthe palms and soles G Nlce$ated
M ate$ $ash -- coppe$-colo$ed,6at o$ !umpy $ash on the ace,
palms, and soles
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Sy$pto$ati +arly CS
the$ ea$ly maniestations
include hepatosplenome)aly+100=, Oaundice, anemia
3etaphyseal dyst$ophy andpe$iostitis oten a$e noted on$adio)$aphs at !i$th PQ?seudopa$alysis
.on)enital syphilis ea$ly
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.on)enital syphilis - ea$lye(idence o inection - !ullae and
(esicula$ $ash
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3ultiple, punched out, pale, !liste$edlesions, with associated desuamation
o palms & planta$s
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"nt$ao$al mucous patches & acials2in lesions
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Seconda$y lesions on eet Lesions frst appeared during 4th week
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Late)onset CS
e(elop $om sca$$in) $elatedto ea$ly inection
.an !e p$e(ented !y t$eatmentwithin $st E months
.an appea$ as late as D0 yea$sate$
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Late)onset CS
3aniestations include neu$osyphilisand in(ol(ement o teeth, !ones,eyes, and /th c$anial ne$(e
4A)A %$ontal !ossin), sho$t mailla,hi)h palatal a$ch, Hutchinson t$iad,
saddle nose, and pe$io$al ssu$e+Lha)ades F !acte$ial inection os2in lesions
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Hutchinson t$iad
1A eaness +10 D0 yea$s
:A Hutchinsons teeth F
cent$ally notched, widely-spaced pe)-shaped uppe$cent$al inciso$s
EA "nte$stitial Ue$atitis G!lindness +5-:0 yea$s
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Notched incisors known asHutchinson’s teeth
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3o$i!und new!o$n with .S
$al s2in lesions and saddle nose
3etaphyseal osteomyelitis
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3etaphyseal osteomyelitisLadiolucent distal $adius & ulna with cuppin)
distal ulna
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steochond$itis o emu$ &ti!ia
1 ld .l i l >i ! V i
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1-m-old A .lassical >im!e$)e$Vs si)n odest$ucti(e metaphysitis in(ol(in) medial
aspects o distal emo$a and p$oimal ti!ae
'Sa!e$ shins* F
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Sa!e$ shins Fsteope$iostitis Ti!ia
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"nte$stitial 2e$atitis
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Possi-le Co$pliations
Blindness
eaness
%acial deo$mity
7eu$olo)ical p$o!lems
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La-s
eniti(e dia)nosis
1A By di$ect (isuali@ation ospi$ochetes usin) da$2eldmic$oscopy
:A $ di$ect 6uo$escent anti!ody testso lesion eudate o$ tissue+?lacentaN.
-Helpful early in the disease, prior to development of seroreactivity
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Serologi tests
- ?$esumpti(e dia)nosis can !e madeusin)
- 7ont$eponemal + %alse P in medical
conditions- T$eponemal +%alseP in othe$
spi$ochetal iseases
G So use o only one type is insuWcient
- " nont$eponemal test is PGcon$mato$y testin) is pe$o$medwith a specic t$eponemal test
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Nontrepone$al test
KL +Kene$eal iseaseLesea$ch a!o$ato$y
L?L +Lapid plasma $ea)in
8LT +8utomated $ea)in test)
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Nontrepone$al test
- Nsed o$ sc$eenin) +sensiti(e !ut notspecic
- "nepensi(e, pe$o$med $apidly, and
p$o(ide uantitati(e $esultsG helpul indicato$s o disease acti(ity &
monito$ t$eatment $esponse- 3easu$es 8! di$ected a)ainst lipoidal
8) $om TA ?allidum, 8! inte$actionwith host tissues o$ !oth
- 7onspecic 8! de(elop D-/ wee2sollowin) inection
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Nontrepone$al test
False negative
- Early primary S-
atent ac!uired S- ate CS- Prozone phenomenon
False "ositive- #iral infection $ E%#,
Hepatitis, #aricela,&easles)
- ymphoma- '%- &alaria- Endocarditis- C' diseases- "regnancy- (# drugs- harton *elly
contamination in cordsamples
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Nontrepone$al test
8ny $eacti(e 7T test must !e con$med!y T$eponemal test to eclude alsepositi(e
T$eatment should not !e delayed isymptomatic o$ at hi)h $is2 o inection
3onito$
- Sustained D old X7T test tite$ ate$
t$eatmentG 8deuate t$eatment
- Sustained Y Le-inection o$ $elapse
N t l t t
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Nontrepone$al test New-orn Dile$$a
Testin) o new!o$n oten is p$o!lematic!ecause ")# anti!ody may !e a $e6ectiono mate$nal $athe$ than inant inection
Nnless 7T tite$ is much hi)he$ in !a!ythan in mothe$ G u se$olo)y o(e$ 1st ;
months o lie, when mate$nal ")# is lost,would !e $eui$ed to ma2e a dia)nosis
i.e. Loosing preious ti$e in treat$ent initiation
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#repone$al Spei #est
T pallidum immobiliation (TP;
<luorescent treponemal antibodyabsorption (<TA*A=$
8icrohemagglutination assay forantibodies to T pallidum (8>A*TP)
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#repone$al Spei #est
0onfirm ? nontreponemal reaginic test
@emain positive for life
i.e. @esult do not correlate with disease
activity and tests are not 2uantified
<alse ? reactions:
Bther spirochetal diseases (e.g.! yaws!
pinta! leptospirosis! rat*bite fever!
relapsing fever! Cyme disease
Cere-rospinal Flui"
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Cere-rospinal Flui" nalysis
0$< D@C
0ould be negative and still develop signs
of neurosyphilis Therefore! all those
with presumptive 0$ should be treated
A non2uantitative D@C test is the only
serologic test that should be performed
on 0$<
+ther test like F'-%S are less specific on CSF samples
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CBC
.S cha$acte$i@ed !y anemia,th$om!ocytopenia, and eithe$
leu2openia o$ leu2ocytosis
4(idence o .oom!s-ne)ati(e
hemolytic anemia o$ aleu2emoid $eaction may !ep$esent
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%$aging Stu"ies
.ZL
- Syphilitic pneumonia iscommon in .S - %lufy difuse inlt$ate
'pneumonia al!a*
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%$aging Stu"ies
on) !one $adio)$aphy
– 95= o symptomatic inants and:0= o asymptomatic
– 3ultiple sites o osteochond$itis atw$ists, el!ows, an2les and 2nees
and pe$iostitis o lon) !ones– The lowe$ et$emities almost
always afected
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%$aging Stu"ies
7eu$o$adio)$aphy
- %indin)s nonspecic
- 3ay mimic he$pes simple(i$us
- 3L" may $e(eal ce$e!$al
hype$t$ophy andhype$intensity in the tempo$allo!es
CDC
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CDCNew-orn +valuation
The dia)nosis o .S iscomplicated !y the t$ans
placental t$anse$ o mate$nalnont$eponemal andt$eponemal ")# 8!s to etus
I 3a2in) inte$p$etation o$eacti(e se$olo)ic tests o$ .SdiWcult
CDC
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CDCNew-orn +valuation
4(aluation should include
1A 3ate$nal H syphilis includin) t type& adeuacy !eo$e and du$in) thep$e)nancy
:A ?4 o new!o$n
EA [uantitati(e 7T & T tests
DA .B., lon) !one -$ays, .S% +KL, cellcount, p$otein, and .ZL ando$ %T
5A ?atholo)ic eamination o placenta o$um!ilical co$d usin) specic 6uo$escentantit$eponemal anti!ody stainin)
CDC
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CDCNew-orn +valuation
8 p$esumpti(e dia)nosis, which $esultsin t, is made i !a!y has P se$olo)ictest
and any o ollowin)1A .ompati!le ndin)s on ?4
:A .S% a!nA +P KL, Y >B., o$ Yp$otein
EA steitis on -$ay lon) !ones
DA ?lacentitis5A 7T test D C than mate$nal
;A ?ositi(e %T8-8BS-19S ")3 anti!ody
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#reat$ent
"K ?enicillin # is the d$u) o choiceo$ all sta)es o syphilis includin) .S
"nants
- 100,000 - 150,000 N2)d "K [1: \dA then [ / to complete 10 days
- $ ?$ocaine ?enicillin # 50,000N2)d "3 once o$ 10 days +adequateCSF conc. may not be achieved)
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#reat$ent
"ndications
1A " new!o$n meets any o c$ite$ia
:A " mothe$ was t$eated ] D wee2sp$io$ to deli(e$y
EA " mothe$ t$eated with othe$ than
penicillinDA " mate$nal tite$s su))est
inadeuate $esponse to t$eatment!eo$e o$ ea$ly in p$e)nancy
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Syphilis %n Pregnany
"n communities in which $is2 o$ .S ishi)h G se$olo)ic testin) and a seualhisto$y also should !e o!tained at :/
wee2s )estation and at deli(e$y
T$eat all p$e)nant patients withpenicillin, $e)a$dless o the sta)e op$e)nancy
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Syphilis %n Pregnany
E doses o !en@athine penicillin
+:AD million N "3 at 1-wee2inte$(als
7o p$o(en alte$nati(e t$eatment o$patient alle$)ic to penicillin
iAeA 4$yth$omycin o$ patient alle$)ic topenicillin is not $elia!le t$eatment o$etus
%n(ants During the First 'onth o(Li(
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Li(e
The ollowin) scena$ios
desc$i!e the e(aluation andt$eatment o inants o$con)enital syphilis
Senario %n(ants with proven or highly pro-a-le "isease
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%n(ants with proven or highly pro-a-le "isease
an"
8!no$mal ?4 consistent with.S
Se$um uantitati(e 7T tite$D C mothe$s tite$ o$
P da$2eld o$ 6A a!A test o!ody 6uids
Senario %n(ants with proven or highly pro-a-le "isease
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%n(ants with proven or highly pro-a-le "isease
an"
1eo$$en"e"+valuation
.S% analysis o$KL, cell count &p$otein
.B. wA difA& ?count
the$ tests asclinically indicated+ lon)-!one -$ays,.ZL, %T, HNS,ophthalmolo)ic
eam, and B84L
1eo$$en"e"1egi$ens
8ueous c$ystallinepenicillin #50,000 N2)dose "K[ 1: h$sA $st \ and [ / h$sthe$eate$ o$ a total
o 10 daysL
?$ocaine penicillin #50,000 units2)dose"3 in a sin)le dailydose o$ 10 days
Senario 2N l P+ 3 titi N# tit 6
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Nor$al P+ 3 seru$ 4uantitive N# titer 5 6 7
$aternal titer
3othe$ not inadeuatelyt$eated, o$ no documentation
3othe$ was t$eated withe$yth$omycin o$ othe$nonpenicillin $e)imen o$
3othe$ $ecei(ed t$eatment ]D wee2s !eo$e deli(e$y
Senario 2
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Senario 2Nor$al P+ 3 seru$ 4uantitive N# titer 5 6 7
$aternal titer
1eo$$en"e"+valuation
.S% analysis o$KL, cell count,and p$otein
.B. wA difA and?T count
on)-!one Z-$ays
1eo$$en"e"1egi$ens
Aqueous crst! penici""in #
50$000 u!%kg%dose &' ( 1)hrs during the 1st * +,Land ( hrs therea.ter .ora tota" o. 10 das
, rocaine penici""in #
50$000 units%kg%dose &2 ina sing"e dai" dose .or 10das
, enathine penici""in #
50$000 units%kg%dose &2 ina sing"e dose
Senario
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Senario Nor$al P+ 3 seru$ 4uantitive N# titer 5 6 7
$aternal titer
3othe$ was t$eated du$in)p$e)nancy, tA was app$op$iate o$the sta)e o inection, andt$eatment was administe$ed C Dwee2s !eo$e deli(e$y^AA and
3othe$ has no e(idence o$einection o$ $elapse
Senario
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Senario Nor$al P+ 3 seru$ 4uantitive N# titer 5 6 7
$aternal titer
1eo$$en"e" +valuation
_
7o e(aluation$eui$ed
1eo$$en"e"1egi$en
_Ben@athine
penicillin # 50$000units%kg%dose &2 in asing"e dose
Senario 6
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Senario 6Nor$al P+ 3 seru$ 4uantitive N# titer 5 6 7
$aternal titer
3othe$s t$eatment was adeuate!eo$e p$e)nancy^A and
3othe$s 7T tite$ $emained lowand sta!le !eo$e, du$in)
p$e)nancy and at deli(e$y +KL]1:` L?L ]1D
Senario 6
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Senario 6Nor$al P+ 3 seru$ 4uantitive N# titer 5 6 7
$aternal titer
1eo$$en"e"+valuation
_
7o e(aluation$eui$ed
1eo$$en"e" 1egi$en
_
7o t$eatment$eui$ed
8/18/2019 Syphilis. 1Dr Frewan Neo
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9utloo! :Prognosis;
"nected ea$ly in p$e)nancy I still!o$n
T$eatment o epectant mothe$ X $is2 o
.S
Ba!ies who !ecome inected whenpassin) th$ou)h !i$th canal ha(e !ette$
outloo2
eath $om .S is usually th$ou)hpulmona$y hemo$$ha)e
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References
www!cdc!go%67+%67A76%fgs!gi.
,eriew o. 7,8H in.ections9 :aren ; <ohnson$ 2+!=ptodate )00>
;ar" congenita" sphi"is9 A?eeta 6ingh$ 26 26c$@:aren 6uther"and$ N 6cN$ onita Lee$ 2+ 26c-puB?ed
8ongenita" sphi"is re-e?erging!6i??s &$ routet N!