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__NOTOC__
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{{SI}}
{{CMG}} {{AE}} {{AKI}}
{{SK}} Congenital lues; fetal syphilis


==Overview==
==Overview==
Congenital [[Congenital syphilis|Syphilis]] is caused by [[Treponema pallidum]], its transmitted to the [[fetus]] in utero from an infected mother via the [[placenta]]. The severity of the disease is dependent on the stage of maternal infection and the duration of exposure to the [[fetus]]. Transmission is typically in the [[second trimester]] and the highest rates of transmission are seen in women with [[primary syphilis]]. The rates of transmission decrease with the increasing duration of the maternal infection, as the concentration of [[spirochetes]] in the blood stream decreases. [[Syphilis]] infection to the fetus in utero can result in [[stillborn]], [[miscarriage]] and a live birth with severe manifestations of [[hydrops]]. [[Prenatal screening]] for [[syphilis]] during the [[first trimester]] is recommended to all pregnant women and adequate treatment with penicillin prevents the transmission to the [[fetus]].
==Historical Perspective==
*In the 19th century congenital syphilis was believed to be transmitted during conception by the father’s sperm, during delivery in the birth canal, or from infected milk or breasts.<ref name="Obladen2013">{{cite journal|last1=Obladen|first1=Michael|title=Curse on Two Generations: A History of Congenital Syphilis|journal=Neonatology|volume=103|issue=4|year=2013|pages=274–280|issn=1661-7819|doi=10.1159/000347107}}</ref>
*In 1905, Schaudinn and Hoffmann identified Spirochaeta  pallida.
*Transplacental transmission from an asymptomatic infected mother was first described in 1906.<ref name="urlGuidelines for the Prevention and Control of Congenital Syphilis">{{cite web |url=https://www.cdc.gov/mmwr/preview/mmwrhtml/00026330.htm |title=Guidelines for the Prevention and Control of Congenital Syphilis |format= |work= |accessdate=}}</ref>
*In 1943, Lentz and Ingraham reported penicillin as treatment for congenital syphilis.
*In 2006, the WHO launched a global effort to eliminate congenital syphilis.


==Classification==
Congenital Syphilis is classified based on the timing of appearance of signs and symptoms into:<ref name="pmid23919053">{{cite journal| author=Balaji G, Kalaivani S| title=Observance of Kassowitz law-late congenital syphilis: Palatal perforation and saddle nose deformity as presenting features. | journal=Indian J Sex Transm Dis | year= 2013 | volume= 34 | issue= 1 | pages= 35-7 | pmid=23919053 | doi=10.4103/0253-7184.112869 | pmc=3730472 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23919053  }} </ref>
*'''Early congenital Syphilis:''' If the [[signs]] and [[symptoms]] are identified in children aged less than 2 years. It is usually diagnosed in new born or in the first few weeks after [[birth]].<ref name="pmid25079189">{{cite journal| author=Cavagnaro S M F, Pereira R T, Pérez P C, Vargas Del V F, Sandoval C C| title=[Early congenital syphilis: a case report]. | journal=Rev Chil Pediatr | year= 2014 | volume= 85 | issue= 1 | pages= 86-93 | pmid=25079189 | doi=10.4067/S0370-41062014000100012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25079189  }} </ref>
*'''Late congenital Syphilis:''' If the [[signs]] and [[symptoms]] of the disease are identified in children aged more than 2 years. The signs are usually non-specific and more than half the children are asymptomatic. They can present with [[interstitial keratitis]], [[Sensorineural hearing loss|sensorineura]]<nowiki/>l deafness or [[Clutton's joints|clutton's]] joints.
*'''Stigmata:''' These are the scars resulting from early or late [[congenital syphilis]]. The features of stigmata in early congenital syphilis include [[saddle nose]] deformity, [[Hutchinson's teeth|Hutchinson's]] teeth, rhagades (linear scars at the angles of the mouth and nose result from bacterial infection of skin lesions), choriod scarring and [[onychia]]. Stigmata secondary to late congenital syphilis include perforation of the [[palate]], corneal opacities, [[optic atrophy]] and [[Periosteal reaction|periosteal]] changes of [[tibia]].


==Causes==
==Causes==
The causative pathogen for [[Congenital syphilis]] [[Treponema pallidum]].


==Pathophysiology==
==Classification==
 
===Pathogenesis===
*Transmission to the [[fetus]] is [[transplacental]], it can also occur during [[delivery]] in the presence of maternal [[Genital area|genital]] lesions.<ref name="pmid11438902">{{cite journal| author=Wicher V, Wicher K| title=Pathogenesis of maternal-fetal syphilis revisited. | journal=Clin Infect Dis | year= 2001 | volume= 33 | issue= 3 | pages= 354-63 | pmid=11438902 | doi=10.1086/321904 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11438902  }} </ref><ref name="pmid27333146">{{cite journal| author=Domingues RM, Leal Mdo C| title=[Incidence of congenital syphilis and factors associated with vertical transmission: data from the Birth in Brazil study]. | journal=Cad Saude Publica | year= 2016 | volume= 32 | issue= 6 | pages=  | pmid=27333146 | doi=10.1590/0102-311X00082415 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27333146  }} </ref><ref name="pmid16362988">{{cite journal| author=Peeling RW, Hook EW| title=The pathogenesis of syphilis: the Great Mimicker, revisited. | journal=J Pathol | year= 2006 | volume= 208 | issue= 2 | pages= 224-32 | pmid=16362988 | doi=10.1002/path.1903 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16362988  }} </ref>
*The risk of transmission to the [[fetus]] is dependent on the stage of the maternal disease(dependent on the [[Spirochaete|spirochete]] concentration in the blood stream) and the duration of exposure to the [[fetus]] in utero.<ref name="pmid15356936">{{cite journal| author=Berman SM| title=Maternal syphilis: pathophysiology and treatment. | journal=Bull World Health Organ | year= 2004 | volume= 82 | issue= 6 | pages= 433-8 | pmid=15356936 | doi= | pmc=2622860 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15356936  }} </ref>
*The risk of [[vertical transmission]] of syphilis from an infected untreated mother decreases as maternal disease duration progresses: transmission risk of 70–100% for primary syphilis and 40% for early latent syphilis to 10% for late latent disease. The variation in the percentages with the duration of infection is due to the concentration of spirochetes in the blood stream, which decrease with the duration of maternal syphilis infection.<ref name="pmid10858706">{{cite journal |vauthors=Genç M, Ledger WJ |title=Syphilis in pregnancy |journal=Sex Transm Infect |volume=76 |issue=2 |pages=73–9 |year=2000 |pmid=10858706 |pmc=1758294 |doi= |url=}}</ref>
*Kassowitz's law describes the an inverse relationship of interval between the disease and pregnancy. Longer the interval between infection and pregnancy more benign is the outcome.<ref name="pmid23919053">{{cite journal| author=Balaji G, Kalaivani S| title=Observance of Kassowitz law-late congenital syphilis: Palatal perforation and saddle nose deformity as presenting features. | journal=Indian J Sex Transm Dis | year= 2013 | volume= 34 | issue= 1 | pages= 35-7 | pmid=23919053 | doi=10.4103/0253-7184.112869 | pmc=3730472 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23919053  }} </ref>
*Transmission of infection typically takes place between the 16th and 28th week of pregnancy, however the transmission can be as early as the [[first trimester]] of [[pregnancy]].<ref name="pmid56895">{{cite journal |vauthors=Harter C, Benirschke K |title=Fetal syphilis in the first trimester |journal=Am. J. Obstet. Gynecol. |volume=124 |issue=7 |pages=705–11 |year=1976 |pmid=56895 |doi= |url=}}</ref
 
===Microscopic Pathology===
*Skin lesion on histopahological exam demonstrate perivascular infiltration by lymphocytes, plasma cells and histiocytes, with endarteritis and extensive fibrosis.
 
==Risk Factors==
Risk factors for congenital syphilis include all the risk factors which predispose a pregnant woman to have syphilis infection:<nowiki><ref name="pmid2356911">{{cite journal| author=Rolfs RT, Goldberg M, Sharrar RG| title=Risk factors for syphilis: cocaine use and prostitution. | journal=Am J Public Health | year= 1990 | volume= 80 | issue= 7 | pages= 853-7 | pmid=2356911 | doi= | pmc=1404975 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2356911  }} </ref><ref name="pmid17675391">{{cite journal| author=Zhou H, Chen XS, Hong FC, Pan P, Yang F, Cai YM et al.| title=Risk factors for syphilis infection among pregnant women: results of a case-control study in Shenzhen, China. | journal=Sex Transm Infect | year= 2007 | volume= 83 | issue= 6 | pages= 476-80 | pmid=17675391 | doi=10.1136/sti.2007.026187 | pmc=2598725 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17675391  }} </ref><ref name="pmid15247352">{{cite journal| author=Hook EW, Peeling RW| title=Syphilis control--a continuing challenge. | journal=N Engl J Med | year= 2004 | volume= 351 | issue= 2 | pages= 122-4 | pmid=15247352 | doi=10.1056/NEJMp048126 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15247352  }} </ref><ref name="pmid16205297">{{cite journal| author=Buchacz K, Greenberg A, Onorato I, Janssen R| title=Syphilis epidemics and human immunodeficiency virus (HIV) incidence among men who have sex with men in the United States: implications for HIV prevention. | journal=Sex Transm Dis | year= 2005 | volume= 32 | issue= 10 Suppl | pages= S73-9 | pmid=16205297 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16205297  }} </ref><ref name="pmid25514173">{{cite journal| author=Solomon MM, Mayer KH| title=Evolution of the syphilis epidemic among men who have sex with men. | journal=Sex Health | year= 2015 | volume= 12 | issue= 2 | pages= 96-102 | pmid=25514173 | doi=10.1071/SH14173 | pmc=4470884 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25514173  }} </ref><ref name="pmid24927712">{{cite journal| author=Hakre S, Arteaga GB, Núñez AE, Arambu N, Aumakhan B, Liu M et al.| title=Prevalence of HIV, syphilis, and other sexually transmitted infections among MSM from three cities in Panama. | journal=J Urban Health | year= 2014 | volume= 91 | issue= 4 | pages= 793-808 | pmid=24927712 | doi=10.1007/s11524-014-9885-4 | pmc=4134449 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24927712  }} </ref><ref name="newell">Newell, J., et al. "A population-based study of syphilis and sexually transmitted disease syndromes in north-western Tanzania. 2. Risk factors and health seeking behaviour." Genitourinary medicine 69.6 (1993): 421-426.</ref>
*Inadequate antenatal care
*Multiple sexual partners
*Prostitution
*Illicit drug use
*Unprotected sex
*Residence in highly prevalent areas
*[[Human Immunodeficiency Virus (HIV)|HIV]] infection
*Presence of other [[STI]]<nowiki/>s
*Previous history of STIs
*Intravenous drug use
*Health care professionals who are predisposed to occupational risk
*Low socioeconomic status
 
==Screening==
Effective prevention and detection of congenital syphilis depends on the identification of syphilis in pregnant women and screening is a key component to decrease the incidence of congenital syphilis. The recommendations for screening are as follows:
{| border="1"
|-
!
!'''Screening Recommendations'''
|-
!'''Timing of Screening'''
|Test all pregnant women at the first prenatal visit.                                                                                                               
|-
!'''Screening Tests'''
|
*Nontreponemal tests commonly used for initial screening include:
**Venereal Disease Research Laboratory [[(VDRL)]]
**Rapid Plasma Reagin (RPR)
*Confirmatory tests include:
**Fluorescent treponemal antibody absorbed [[(FTA-ABS)]]
**Treponema pallidum particle agglutination [[(TPPA)]]
|-
!'''High Risk Population'''
|
*Most organizations recommend testing high-risk women again during the [[third trimester]] and at [[delivery]].<br>Groups at increased risk include:
**Uninsured women
**Women living in poverty
**Sex workers
**Illicit drug users
**Those diagnosed with other [[sexually transmitted diseases]] (STDs)
**Other women living in communities with high [[syphilis]] [[morbidity]]
|}
 
==Epidemiology, Demographics==
===Incidence===
*It is estimated that every year 2 million [[pregnant]] women are infected with [[syphilis]] every year.
*In 2005, WHO reported that 460,000 [[abortions]] or [[still birth]] and 270,000 cases of [[congenital syphilis]] every year can be attributed to maternal [[syphilis]].
*The [[incidence]] of [[congenital syphilis]] in United States in the year 2014 is 11.6 cases per 100,000 [[live births]]. The incidence of [[congenital syphilis]] has increased when compared to the numbers from 2008 to 2012 and the change is attributed to the increase in the number of women with primary and secondary [[syphilis]].<ref name="pmid25487961">{{cite journal| author=Peterman TA, Su J, Bernstein KT, Weinstock H| title=Syphilis in the United States: on the rise? | journal=Expert Rev Anti Infect Ther | year= 2015 | volume= 13 | issue= 2 | pages= 161-8 | pmid=25487961 | doi=10.1586/14787210.2015.990384 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25487961  }} </ref><ref name="pmid26562206">{{cite journal| author=Bowen V, Su J, Torrone E, Kidd S, Weinstock H| title=Increase in incidence of congenital syphilis - United States, 2012-2014. | journal=MMWR Morb Mortal Wkly Rep | year= 2015 | volume= 64 | issue= 44 | pages= 1241-5 | pmid=26562206 | doi=10.15585/mmwr.mm6444a3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26562206  }} </ref>
 
===Race===
*[[Congenital syphilis]] is ten times more [[prevalent]] in black population compared to whites and three times more common in blacks compared to Hispanics.
 
==Natural History, Complications and Prognosis==
===Natural History===
[[Syphilis]] is a [[sexually transmitted disease]] and is more [[prevalent]] in [[high risk]] population. Women with [[syphilis]] infection can transmit the [[infection]] to the [[fetus]] in utero resulting in a wide spectrum of outcomes. The risk of transmission is higher in [[pregnant]] women who do not undergo regular [[antenatal screening]] or in women who are untreated or adequately treated during the period of [[gestation]]. The risk of transmission to the fetus is dependent on the stage of [[syphilis]] infection in the mother (primary, secondary, tertiary or latent), duration of maternal infection and the exposure to [[fetus]] in utero. The transmission of infection typically occurs during the [[second trimester]] but early [[transmission]] also occurs. [[Syphilis]] can complicate the outcome of [[pregnancy]] and is dependent on the severity of infection in the [[fetus]], severe infection has adverse outcomes in the [[new born]].<ref name="pmid6340889">{{cite journal| author=Charles D| title=Syphilis. | journal=Clin Obstet Gynecol | year= 1983 | volume= 26 | issue= 1 | pages= 125-37 | pmid=6340889 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6340889  }} </ref><ref name="QinFeng2014">{{cite journal|last1=Qin|first1=Jia-Bi|last2=Feng|first2=Tie-Jian|last3=Yang|first3=Tu-Bao|last4=Hong|first4=Fu-Chang|last5=Lan|first5=Li-Na|last6=Zhang|first6=Chun-Lai|last7=Yang|first7=Fan|last8=Mamady|first8=Keita|last9=Dong|first9=Willa|title=Risk Factors for Congenital Syphilis and Adverse Pregnancy Outcomes in Offspring of Women With Syphilis in Shenzhen, China|journal=Sexually Transmitted Diseases|volume=41|issue=1|year=2014|pages=13–23|issn=0148-5717|doi=10.1097/OLQ.0000000000000062}}</ref>
 
===Complications===
*Severe infection in the [[fetus]] can result in [[spontaneous abortion]], [[stillbirth]], non-immune [[hydrops]], [[intrauterine growth restriction]], and [[perinatal]] death, and other serious sequelae in liveborn.<ref name="pmid24275265">{{cite journal| author=Cohen SE, Klausner JD, Engelman J, Philip S| title=Syphilis in the modern era: an update for physicians. | journal=Infect Dis Clin North Am | year= 2013 | volume= 27 | issue= 4 | pages= 705-22 | pmid=24275265 | doi=10.1016/j.idc.2013.08.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24275265  }} </ref>
 
===Prognosis===
*Adequate treatment of infected mother during the period of [[gestation]] can prevent the [[transmission]] of disease significantly and treatment of the mother with one dose of [[pencillin]] is proven to improve outcomes in the [[fetus]].<ref name="pmid12232835">{{cite journal |vauthors=Watson-Jones D, Gumodoka B, Weiss H, Changalucha J, Todd J, Mugeye K, Buvé A, Kanga Z, Ndeki L, Rusizoka M, Ross D, Marealle J, Balira R, Mabey D, Hayes R |title=Syphilis in pregnancy in Tanzania. II. The effectiveness of antenatal syphilis screening and single-dose benzathine penicillin treatment for the prevention of adverse pregnancy outcomes |journal=J. Infect. Dis. |volume=186 |issue=7 |pages=948–57 |year=2002 |pmid=12232835 |doi=10.1086/342951 |url=}}</ref>
*In [[newborns]] and [[infants]] with [[congenital syphilis]] treatment with [[penicillin]] has good [[prognosis]] with normal development.<ref name="pmid21639965">{{cite journal| author=Caddy SC, Lee BE, Sutherland K, Robinson JL, Plitt SS, Read R et al.| title=Pregnancy and neonatal outcomes of women with reactive syphilis serology in Alberta, 2002 to 2006. | journal=J Obstet Gynaecol Can | year= 2011 | volume= 33 | issue= 5 | pages= 453-9 | pmid=21639965 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21639965  }} </ref>
 
==Diagosis==
 
===History and Symptoms===
A combination of maternal risk factors and symptoms in the baby are essential to suspect [[congenital syphilis]]. The most common symptoms in the new born include: <ref name="pmid3288424">{{cite journal| author=Wendel GD| title=Gestational and congenital syphilis. | journal=Clin Perinatol | year= 1988 | volume= 15 | issue= 2 | pages= 287-303 | pmid=3288424 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3288424  }}</ref> <ref name="pmid16649151">{{cite journal| author=Kremenová S, Zákoucká H, Kremen J| title=[Issues of congenital syphilis in the past twenty years. II. Clinical picture]. | journal=Klin Mikrobiol Infekc Lek | year= 2006 | volume= 12 | issue= 2 | pages= 51-7 | pmid=16649151 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16649151  }} </ref>
*[[Skin rash]] occurs in 70% of affected cases.It appears as small [[blister]]s on the palms and soles, copper-colored, flat or bumpy rash on the face, palms, and soles
*Yellowish discoloration of the skin
*[[Abdominal distension]]
*Generalized [[edema]]
*Irritability
*[[Low birth weight]]
*[[Failure to thrive]]
*[[Fever]]
*Difficulty breathing
Late Syphilis: It is diagnosed in children aged greater than 2 years. The symptoms are non specific and present with [[skin rash]], [[rhinitis]] and features of stigmata.
 
===Physical Examination===
The physical examination findings suggestive of [[congenital syphilis]] include:<ref name="EwingRoberts1985">{{cite journal|last1=Ewing|first1=C I|last2=Roberts|first2=C|last3=Davidson|first3=D C|last4=Arya|first4=O P|title=Early congenital syphilis still occurs.|journal=Archives of Disease in Childhood|volume=60|issue=12|year=1985|pages=1128–1133|issn=0003-9888|doi=10.1136/adc.60.12.1128}}</ref>
*[[Vesiculobullous]] or maculopapular rash occurring on the palms and soles is present in 70% of the children with congenital syphilis. Other patterns of rash such as [[condylomata lata]] lesions, annular lesions, and [[erythema multiforme ]]-like targetoid lesions are present in affected infants.<ref name="FerreiraCorreia2016">{{cite journal|last1=Ferreira|first1=Sara Tavares|last2=Correia|first2=Cátia|last3=Marçal|first3=Monica|last4=Tuna|first4=Madalena Lopo|title=Skin rash: a manifestation of early congenital syphilis|journal=BMJ Case Reports|year=2016|pages=bcr2016216148|issn=1757-790X|doi=10.1136/bcr-2016-216148}}</ref>
*[[Low birth weight]] with signs of [[prematurity]]
*Nonimmune [[hydrops]] : It is characteristic of [[congenital syphilis]] and the features of non-immune [[hydrops]] include [[ascites]], [[pericardial effusion]], [[pleural effusion]] and skin [[edema]], however [[Rh incompatability]] should be ruled out as a cause of [[hydrops]].<ref name="pmid8822333">{{cite journal| author=Barron SD, Pass RF| title=Infectious causes of hydrops fetalis. | journal=Semin Perinatol | year= 1995 | volume= 19 | issue= 6 | pages= 493-501 | pmid=8822333 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8822333  }} </ref>
**Hutchinson's Triad: It is the common presentation of congenital syphilis and includes [[deafness]], [[Hutchinson's teeth]] (centrally notched, widely-spaced peg-shaped upper central [[incisors]]), and interstitial [[keratitis]].
*[[Jaundice]]
*[[Hepatosplenomegaly]]
*[[Rhinitis]]
*[[Lymphadenopathy]]
*[[Pseudoparalysis]] of an extremities
*Sabre [[shin]]s: It is a sharp-edged anteriorly convex [[tibia]]
 
===Laboratory Findings===
====Prenatal Diagnosis====
*Detection of [[IgM]] [[antibodies]] aganist [[T.pallidum]] in the blood collected by [[chordocentesis]].<ref name="pmid1923218">{{cite journal |vauthors=Wendel GD, Sánchez PJ, Peters MT, Harstad TW, Potter LL, Norgard MV |title=Identification of Treponema pallidum in amniotic fluid and fetal blood from pregnancies complicated by congenital syphilis |journal=Obstet Gynecol |volume=78 |issue=5 Pt 2 |pages=890–5 |year=1991 |pmid=1923218 |doi= |url=}}</ref><ref name="pmid26753496">{{cite journal| author=Park JY, Han GH, Kwon DY, Hong HR, Seol HJ| title=Prenatal diagnosis of congenital syphilis presenting with transient pleural effusion in the fetus: a case report and rising incidence of congenital syphilis in South Korea. | journal=Clin Exp Obstet Gynecol | year= 2015 | volume= 42 | issue= 6 | pages= 822-4 | pmid=26753496 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26753496  }} </ref>
*[[PCR]] of the [[amniotic fluid]] to detect [[T. pallidum]] [[DNA]].<ref name="MullerEwert2006">{{cite journal|last1=Muller|first1=M|last2=Ewert|first2=I|last3=Hansmann|first3=F|last4=Tiemann|first4=C|last5=Hagedorn|first5=H J|last6=Solbach|first6=W|last7=Roider|first7=J|last8=Nolle|first8=B|last9=Laqua|first9=H|last10=Hoerauf|first10=H|title=Detection of Treponema pallidum in the vitreous by PCR|journal=British Journal of Ophthalmology|volume=91|issue=5|year=2006|pages=592–595|issn=0007-1161|doi=10.1136/bjo.2006.110288}}</ref>
*[[Antenatal]] [[ultrasound]] is commonly done and the findings suggestive of [[congenital syphilis]] include: [[hydrops fetalis]] characterised by scalp oedema, [[placental thickening]], serous cavity effusion, and [[polyhydramnios]]. Other additional findings inlcude [[hepatosplenomegaly]], [[placentomegaly]], non-continuous [[gastrointestinal obstruction]] and dilatation of the [[small bowel]].<ref name="pmid9565220">{{cite journal |vauthors=Levine Z, Sherer DM, Jacobs A, Rotenberg O |title=Nonimmune hydrops fetalis due to congenital syphilis associated with negative intrapartum maternal serology screening |journal=Am J Perinatol |volume=15 |issue=4 |pages=233–6 |year=1998 |pmid=9565220 |doi=10.1055/s-2007-993933 |url=}}</ref><ref name="Russell1974">{{cite journal|last1=Russell|first1=Peter|title=Placental Abnormalities of Congenital Syphilis|journal=American Journal of Diseases of Children|volume=128|issue=2|year=1974|pages=160|issn=0002-922X|doi=10.1001/archpedi.1974.02110270034007}}</ref><ref name="pmid7927729">{{cite journal |vauthors=Riley BS, Oppenheimer-Marks N, Radolf JD, Norgard MV |title=Virulent Treponema pallidum promotes adhesion of leukocytes to human vascular endothelial cells |journal=Infect. Immun. |volume=62 |issue=10 |pages=4622–5 |year=1994 |pmid=7927729 |pmc=303152 |doi= |url=}}</ref>
 
====Postnatal Diagnosis====
*Examination of the [[placenta]] or [[umbilical cord]] using a [[silver  stain]] demonstrates [[spirochetes]] or a [[T. pallidum]] [[PCR]] test can be done.
*The use of [[serological tests]] to identify the infection in infants less than 15 months of age born to infected mothers is not performed as passive transfer of [[IgG]] [[antibodies]] to the [[fetus]] occurs during the [[pregnancy]].
*Other laboratory findings in the [[new born]] include:<ref name="pmid11384701">{{cite journal |vauthors=Hollier LM, Harstad TW, Sanchez PJ, Twickler DM, Wendel GD |title=Fetal syphilis: clinical and laboratory characteristics |journal=Obstet Gynecol |volume=97 |issue=6 |pages=947–53 |year=2001 |pmid=11384701 |doi= |url=}}</ref>
*Elevated [[liver enzymes]]
*[[Leucocytosis]]
*[[Coombs]] negative [[hemolytic anaemia]]
*[[Thrombocytopenia]]
*[[Hypoalbuminemia]]
*[[Hyperbilirubinemia]]
 
===Imaging Studies===
====X-Ray====
*Skeletal survey in a [[still born]], typical [[osseous lesions]] are demonstrated in [[congenital syphilis]].
 
====Long Bone Radiographs====
*Radiographs typically demonstrate bilateral, symmetric, and [[polyostotic]] lesions in [[femur]], [[humerus]], and [[tibia]].<ref name="pmid2584243">{{cite journal| author=Rasool MN, Govender S| title=The skeletal manifestations of congenital syphilis. A review of 197 cases. | journal=J Bone Joint Surg Br | year= 1989 | volume= 71 | issue= 5 | pages= 752-5 | pmid=2584243 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2584243  }} </ref><ref name="pmid8609122">{{cite journal| author=Kocher MS, Caniza M| title=Parrot pseudoparalysis of the upper extremities. A case report. | journal=J Bone Joint Surg Am | year= 1996 | volume= 78 | issue= 2 | pages= 284-7 | pmid=8609122 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8609122  }} </ref>
*Common findings on [[radiographs]] include:
**[[Metaphyseal]] lucent bands
**Symmetric localized demineralization and [[osseous]] destruction of proximal [[tibial]] [[metaphysis]]
**[[Metaphyseal]] serration
 
====Ultrasound====
Antenatal sonographic features include:<ref name="radiop2"> https://radiopaedia.org/articles/in-utero-syphilis-infection. Accessed on September 28th, 2016. </ref><ref name="pmid21844732">{{cite journal| author=Reyna-Figueroa J, Esparza-Aguilar M, Hernández-Hernández Ldel C, Fernández-Canton S, Richardson-Lopez Collada VL| title=Congenital syphilis, a reemergent disease in Mexico: its epidemiology during the last 2 decades. | journal=Sex Transm Dis | year= 2011 | volume= 38 | issue= 9 | pages= 798-801 | pmid=21844732 | doi=10.1097/OLQ.0b013e31821898ca | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21844732  }} </ref>
*Fetal [[hepatosplenomegaly]]
* Placentomegaly
* Fetal [[ascites]]<br>
In severe cases findings include:
*Fetal [[hydrops]]
*Bent fetal long bones
 
====Doppler Studies====
[[Doppler ultrasound]] of the [[uterine]] and [[umbilical]] [[arteries]] show increase in the mean [[systolic]] to [[diastolic]] ratios in mothers infected with [[syphilis]] indicating an increased resistance to [[perfusion]] of the [[placenta]] secondary to [[vasculitis]], placental villitis and obliterative arteritis caused by [[syphilis]].<ref name="Genc2000">{{cite journal|last1=Genc|first1=M.|title=Syphilis in pregnancy|journal=Sexually Transmitted Infections|volume=76|issue=2|year=2000|pages=73–79|issn=13684973|doi=10.1136/sti.76.2.73}}</ref>
 
===Other Diagnostic Studies===
====CSF Analysis====
'''Indications : ''' [[Lumbar puncture]] is indicated in the following situations.<ref name="Phiske2014">{{cite journal|last1=Phiske|first1=MeghanaMadhukar|title=Current trends in congenital syphilis|journal=Indian Journal of Sexually Transmitted Diseases and AIDS|volume=35|issue=1|year=2014|pages=12|issn=0253-7184|doi=10.4103/0253-7184.132404}}</ref>
*If the [[infant]] or [[child]] has [[signs]] and [[symptoms]] of [[congenital Syphilis]].
*If there is no documentation of treatment for [[maternal]] infection during the period of [[gestation]].
*If the mother was treated within 4 weeks of [[delivery]].
*If the mother was inadequately treated or documentation of the treatment is incomplete.
*A four-fold decline in [[titer]] following therapy in the mother is not documented.
'''CSF Findings:'''
*Reactive [[CSF]] [[VDRL]]. <ref name="pmid26042815">{{cite journal| author=Workowski KA, Bolan GA, Centers for Disease Control and Prevention| title=Sexually transmitted diseases treatment guidelines, 2015. | journal=MMWR Recomm Rep | year= 2015 | volume= 64 | issue= RR-03 | pages= 1-137 | pmid=26042815 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26042815  }} </ref>
*[[CSF pleocytosis]](>25 white blood cells [WBC]/microL for infants <1 month)
*Elevated [[CSF]] [[protein]] (>150 mg/dL in term infants <1 month of age and >170 mg/dL in preterm infants <1 month of age)
 
==Treatment==
 
===Medical Therapy===
====Management during Antenatal Period====
{| border="1"
|-
!
!'''CDC Recommendations for management of pregnant woman with Syphilis infection'''
|-
!'''Approach during the Prenatal Period'''
|
*All women should be screened [[Syphilis laboratory tests#Serology|serologically]] for [[syphilis]] early in [[pregnancy]].<ref name=syphilis>https://www.uspreventiveservicestaskforce.org/Page/Document/RecommendationStatementFinal/syphilis-infection-in-pregnancy-screening Accessed on september 27,2016</ref><ref name=cdc2015>http://www.cdc.gov/std/tg2015/references.htm#424 Accessed on September 27, 2016</ref>
*Most states mandate screening at the first prenatal visit for all women;<ref name="pmid11384701">Hollier LM, Harstad TW, Sanchez PJ, Twickler DM, Wendel GD (2001) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11384701 Fetal syphilis: clinical and laboratory characteristics.] ''Obstet Gynecol'' 97 (6):947-53. PMID: [http://pubmed.gov/11384701 11384701]</ref>antepartum screening by [[Syphilis laboratory findings#Nontreponemal test|nontreponemal antibody testing]] is typical, but in some settings, [[Syphilis laboratory findings#Treponemal test|treponemal antibody testing]] is being used.
*[[Pregnant women]] with reactive [[treponemal]] screening tests should have confirmatory testing with [[nontreponemal]] tests with titers to monitor treatment response.
*In populations in which use of [[prenatal care]] is not optimal, [[Rapid plasma reagent|RPR test]] screening and treatment (if the RPR test is reactive) should be performed at the time that pregnancy is confirmed.
*For communities and populations in which the [[prevalence]] of [[syphilis]] is high and for patients at high risk, [[serologic testing]] should be performed twice during the [[third trimester]] (ideally at 28-32 weeks' gestation) and at delivery.
*Any woman who delivers a [[stillborn]] after 20 weeks of [[gestation]] should be tested for [[syphilis]].
*No [[infant]] should leave the hospital without the maternal serologic status having been determined at least once during [[pregnancy]].
*Quantitative maternal [[nontreponemal titer]], especially if >1:8, might be a marker of early infection and [[bacteremia]]. However, risk for fetal infection is still significant in [[pregnant]] women with late [[latent syphilis]] and low titers.
*[[Seropositive]] [[pregnant]] women should be considered infected unless an adequate treatment history is documented clearly in the medical records and sequential [[serologic]] [[antibody]] titers have declined.
*Serofast low [[antibody]] [[titers]] might not require treatment; however, persistent higher titer antibody tests might indicate reinfection, and treatment might be required.
|-
!'''Recommended Regimen for Treatment'''
|
*Pregnant women should be treated with the [[penicillin]] regimen appropriate for their stage of infection.<ref name="urlSexually Transmitted Diseases Treatment Guidelines, 2010">{{cite web|url=http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5912a1.htm |title=Sexually Transmitted Diseases Treatment Guidelines, 2010 |format= |work= |accessdate=2012-12-19}}</ref>
* [[Penicillin]] is effective for preventing maternal transmission to the [[fetus]] and for treating fetal infection.<ref name="pmid9916946">Alexander JM, Sheffield JS, Sanchez PJ, Mayfield J, Wendel GD (1999) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=9916946 Efficacy of treatment for syphilis in pregnancy.] ''Obstet Gynecol'' 93 (1):5-8. PMID: [http://pubmed.gov/9916946 9916946]</ref>Evidence is insufficient to determine optimal, recommended penicillin regimens.<ref name="pmid11686978">Walker GJ (2001) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11686978 Antibiotics for syphilis diagnosed during pregnancy.] ''Cochrane Database Syst Rev''  (3):CD001143. [http://dx.doi.org/10.1002/14651858.CD001143 DOI:10.1002/14651858.CD001143] PMID: [http://pubmed.gov/11686978 11686978]</ref>
|-
!'''Additional Considerations'''
|
*Some evidence suggests that additional therapy can be beneficial for pregnant women in some settings (e.g., a second dose of [[Penicillin#Benzylpenicillin (penicillin G)|benzathine penicillin]] 2.4 million units IM administered 1 week after the initial dose for women who have [[Syphilis pathophysiology#Primary syphilis|primary]], [[Syphilis pathophysiology#Secondary syphilis|secondary]], or [[Syphilis pathophysiology#Latent syphilis|early latent syphilis]]). <ref name="pmid12353207">Wendel GD, Sheffield JS, Hollier LM, Hill JB, Ramsey PS, Sánchez PJ (2002) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=12353207 Treatment of syphilis in pregnancy and prevention of congenital syphilis.] ''Clin Infect Dis'' 35 (Suppl 2):S200-9. [http://dx.doi.org/10.1086/342108 DOI:10.1086/342108] PMID: [http://pubmed.gov/12353207 12353207]</ref>
*When syphilis is diagnosed during the second half of pregnancy, management should include a sonographic fetal evaluation for [[congenital syphilis]], but this evaluation should not delay therapy.
*Sonographic signs of fetal or placental syphilis (i.e., [[hepatomegaly]], [[ascites]], [[hydrops]], [[anemia|fetal anemia]], or a thickened placenta) indicate a greater risk for fetal treatment failure;<ref name="pmid11384701">Hollier LM, Harstad TW, Sanchez PJ, Twickler DM, Wendel GD (2001) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11384701 Fetal syphilis: clinical and laboratory characteristics.] ''Obstet Gynecol'' 97 (6):947-53. PMID: [http://pubmed.gov/11384701 11384701]</ref> such cases should be managed in consultation with obstetric specialists. Evidence is insufficient to recommend specific regimens for these situations.
*Women treated for syphilis during the second half of pregnancy are at risk for [[premature labor]] and/or [[fetal distress]] if the treatment precipitates the [[Jarisch-Herxheimer reaction]].<ref name="pmid2304710">Klein VR, Cox SM, Mitchell MD, Wendel GD (1990) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=2304710 The Jarisch-Herxheimer reaction complicating syphilotherapy in pregnancy.] ''Obstet Gynecol'' 75 (3 Pt 1):375-80. PMID: [http://pubmed.gov/2304710 2304710]</ref> These women should be advised to seek obstetric attention after treatment if they notice any fever, contractions, or decrease in fetal movements.
*[[Stillbirth]] is a rare complication of treatment, but concern for this complication should not delay necessary treatment.
*Pregnant women taking treatment for late latent syphilis should not miss any dose, else she must repeat the whole course of therapy.<ref name="pmid8355931">{{cite journal| author=Nathan L, Bawdon RE, Sidawi JE, Stettler RW, McIntire DM, Wendel GD| title=Penicillin levels following the administration of benzathine penicillin G in pregnancy. | journal=Obstet Gynecol | year= 1993 | volume= 82 | issue= 3 | pages= 338-42 | pmid=8355931 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8355931  }} </ref>
*All patients who have syphilis should be offered testing for HIV infection.
|-
!'''In patients with Penicillin Allergy'''
|
*For treatment of syphilis during pregnancy, no proven alternatives to [[penicillin]] exist.
*Pregnant women who have a history of [[Syphilis medical therapy#Pencillin allergy|penicillin allergy]] should be desensitized and treated with [[penicillin]].
*Oral step-wise penicillin dose challenge or [[Syphilis medical therapy#Pencillin allergy: Penicillin skin test|skin testing]] may be helpful in identifying women at risk for acute allergic reactions.
*[[Tetracycline]] and [[doxycycline]] usually are not used during pregnancy. [[Erythromycin]] and [[azithromycin]] should not be used, because neither reliably cures maternal infection or treats an infected fetus.<ref name="pmid11686978">Walker GJ (2001) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=11686978 Antibiotics for syphilis diagnosed during pregnancy.] ''Cochrane Database Syst Rev''  (3):CD001143. [http://dx.doi.org/10.1002/14651858.CD001143 DOI:10.1002/14651858.CD001143] PMID: [http://pubmed.gov/11686978 11686978]</ref>
*Data are insufficient to recommend [[ceftriaxone]] for treatment of maternal infection and prevention of [[congenital syphilis]].
|-
!'''Pregnant Woman with HIV Infection'''
|
*Placental inflammation from [[congenital]] infection might increase the risk for [[perinatal]] transmission of [[HIV]].
*All [[HIV]]-infected women should be evaluated for [[syphilis]] and receive treatment as recommended.
*Data are insufficient to recommend a specific regimen for HIV-infected pregnant women.
|-
!'''Follow Up'''
|
*Coordinated prenatal care and treatment are vital.
*[[Serologic]] titers should be repeated at 28-32 weeks' gestation and at [[delivery]] as recommended for the disease stage. Providers should ensure that the clinical and antibody responses are appropriate for the patient's stage of disease, although most women will deliver before their serologic response to treatment can be assessed definitively.
*Inadequate maternal treatment is likely if delivery occurs within 30 days of therapy, if clinical signs of infection are present at delivery, or if the maternal [[antibody]] titer at delivery is fourfold higher than the pretreatment titer.
*[[Serologic]] titers can be checked monthly in women at high risk for reinfection or in geographic areas in which the prevalence of [[syphilis]] is high.<ref name="urlSexually Transmitted Diseases Treatment Guidelines, 2010">{{cite web|url=http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5912a1.htm |title=Sexually Transmitted Diseases Treatment Guidelines, 2010 |format= |work= |accessdate=2012-12-19}}</ref>
|}
 
====Management of a Neonate or an Infant with Congenital Syphilis====
The diagnosis of [[congenital syphilis]] can be difficult, as maternal [[nontreponemal]] and [[treponemal]] [[IgG]] [[antibodies]] can be transferred through the [[placenta]] to the [[fetus]], complicating the interpretation of reactive [[serologic]] tests for [[syphilis]] in [[neonates]]. Therefore, treatment decisions frequently must be made on the basis of:
*Identification of [[syphilis]] in the mother
*Adequacy of maternal treatment
*Presence of clinical, laboratory, or [[radiographic]] evidence of [[syphilis]] in the [[neonate]]
*Comparison of maternal (at delivery) and [[neonatal]] [[nontreponemal]] [[serologic]] [[titers]] using the same test, preferably conducted by the same laboratory.
 
====Evaluation and Approach====
*All [[neonates]] born to mothers who have reactive [[nontreponemal]] and [[treponemal]] test results should be evaluated with a quantitative [[nontreponemal]] [[serologic]] test ([[RPR]] or [[VDRL]]) performed on the [[neonate's]] [[serum]], because [[umbilical cord]] blood can become contaminated with maternal blood and yield a [[false-positive result]], and Wharton's jelly within the [[umbilical cord]] can yield a false-negative result.
*Conducting a treponemal test (i.e., TP-PA, FTA-ABS, EIA, or CIA) on neonatal serum is not recommended because it is difficult to interpret.
*Any [[neonate]] at risk for [[congenital syphilis]] should receive a full evaluation and testing for [[HIV]] infection.
*The following scenarios describe the [[congenital syphilis]] evaluation and treatment of [[neonates]] born to women who have reactive [[serologic]] tests for [[syphilis]] during [[pregnancy]]. Maternal history of infection with [[T. pallidum]] and treatment for [[syphilis]] must be considered when evaluating and treating the neonate for [[congenital syphilis]] in most scenarios, except when [[congenital syphilis]] is proven or highly probable.
{| border="1"
|-
!
!'''[[CDC]] Recommendations for management of [[neonates]] with [[congenital Syphilis]]'''
|-
!'''Clinical senario 1'''
|
*[[Infants]] with proven or highly probable disease and with any one of the following :
**An abnormal physical examination that is consistent with [[congenital syphilis]] '''or'''
**A serum quantitative [[non treponemal]] [[serologic]] titer that is fourfold higher than the mother's titer '''or'''
**A positive [[darkfield test]] of body fluid(s).
'''Recommended Evaluation'''
*[[CSF]] analysis for [[VDRL]], cell count, and protein
*[[Complete blood count]] (CBC) and differential and platelet count
*Other tests as clinically indicated (e.g., long-bone radiographs, chest radiograph, liver-function tests, neuroimaging, ophthalmologic examination, and auditory brain stem response)
'''Preferred regimen 1:''' [[Aqueous crystalline penicillin G]] 100,000-150,000 U/kg/day, administered as 50,000 U/kg/dose IV q12h during the first 7 days of life and q8h thereafter for a total of 10 days<br>
'''Preferred regimen 2:''' [[Procaine penicillin G]] 50,000 U/kg/dose IM q24h for 10 days<br>
<small>Note: If more than 1 day of therapy is missed, the entire course should be restarted. Data are insufficient regarding the use of other antimicrobial agents (e.g., [[ampicillin]]). When possible, a full 10-day course of [[penicillin]] is preferred, even if ampicillin was initially provided for possible sepsis. The use of agents other than penicillin requires close serologic follow-up to assess adequacy of therapy. In all other situations, the maternal history of infection with ''T. pallidum'' and treatment for syphilis must be considered when evaluating and treating the infant <small>
|-
!'''Clinical senario 2'''
|
*[[Infants]] who have a normal physical examination and a serum quantitative nontreponemal serologic titer the same or less than fourfold the maternal titer '''and''' with one of the following:
**Mother was not treated or inadequately treated, or has no documentation of having received treatment '''or'''
**Mother was treated with [[erythromycin]] or another non-penicillin regimen '''or'''
**Mother received treatment less than 4 weeks before [[delivery]].
'''Recommended Evaluation'''
*[[CSF]] analysis for [[VDRL]], cell count, and protein
*CBC, differential, and platelet count
*Long-bone [[radiographs]]
'''Preferred regimen 1:''' [[Aqueous crystalline penicillin G]] 100,000-150,000 U/kg/day, administered as 50,000 U/kg/dose IV q12h during the first 7 days of life and q8h thereafter for a total of 10 days<br>
'''Preferred regimen 2:''' [[Procaine penicillin G]] 50,000 U/kg/dose IM q24h for 10 days<br>
'''Preferred regimen 3:''' [[Benzathine penicillin G]] 50,000 U/kg/dose IM single dose<br>
<small>Note: If the mother has untreated early syphilis at delivery, 10 days of parenteral therapy can be considered<br>
Before using the single-dose benzathine penicillin G regimen, the complete evaluation (i.e., CSF examination, long-bone radiographs, and CBC with platelets) must be normal, and follow-up must be certain. If any part of the infant's evaluation is abnormal or not performed, if the CSF analysis is uninterpretable because of contamination with blood, or if follow-up is uncertain, a 10-day course of penicillin G is required. If the neonate's nontreponemal test is nonreactive and the provider determines that the mother's risk of untreated syphilis is low, treatment of the neonate with a single IM dose of benzathine penicillin G 50,000 units/kg for possible incubating syphilis can be considered without an evaluation.<br>
Neonates born to mothers with untreated early syphilis at the time of delivery are at increased risk for congenital syphilis, and the 10-day course of penicillin G may be considered even if the complete evaluation is normal and follow-up is certain.<small>
|-
!'''Clinical senario 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'''
*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 recommended
'''Preferred regimen:''' [[Benzathine penicillin G]] 50,000 U/kg/dose IM single dose
|-
!'''Clinical senario 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'''
*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 recommended
*No treatment is required
*[[Benzathine penicillin G]] 50,000 U/kg IM single dose might be considered, particularly if follow-up is uncertain
|-
!'''Follow up'''
|
*All [[neonates]] with reactive [[nontreponemal]] tests should receive careful follow-up examinations and [[serologic]] testing (i.e., a nontreponemal test) every 2–3 months until the test becomes nonreactive.
*In the [[neonate]] who was not treated because [[congenital syphilis]] was considered less likely or unlikely, [[nontreponemal]] [[antibody]] [[titers]] should decline by age 3 months and be nonreactive by age 6 months, indicating that the reactive test result was caused by passive transfer of maternal [[IgG]] [[antibody]].
*At 6 months, if the [[nontreponemal]] test is nonreactive, no further evaluation or treatment is needed; if the nontreponemal test is still reactive, the infant is likely to be infected and should be treated.
*Treated [[neonates]] that exhibit persistent [[nontreponemal]] test [[titers]] by 6–12 months should be re-evaluated through [[CSF]] examination and managed in consultation with an expert. Retreatment with a 10-day course of a penicillin G regimen may be indicated.
*[[Neonates]] with a negative nontreponemal test at birth and whose mothers were seroreactive at delivery should be retested at 3 months to rule out serologically negative incubating [[congenital syphilis]] at the time of [[birth]].
*[[Neonates]] whose initial [[CSF]] evaluations are abnormal should undergo a repeat [[lumbar puncture]] approximately every 6 months until the results are normal
<small>Note: Treponemal tests should not be used to evaluate treatment response because the results are qualitative and passive transfer of maternal [[IgG]] treponemal antibody might persist for at least 15 months<br>
A reactive [[CSF]] Venereal Disease Research Laboratory (VDRL) test or abnormal CSF indices that persist and cannot be attributed to other ongoing illness requires retreatment for possible neurosyphilis and should be managed in consultation with an expert.<small>
|-
!'''Penicillin Allergy'''
|
*[[Infants]] and [[children]] who require treatment for [[congenital syphilis]] but who have a history of penicillin allergy or develop an allergic reaction presumed secondary to penicillin should be desensitized and then treated with [[penicillin]].
* Data are insufficient regarding the use of other antimicrobial agents (e.g., [[ceftriaxone]]) for [[congenital syphilis]] in [[infants]] and [[children]].
|}
 
{| border="1"
|-
!
!'''[[CDC]] Recommendations for management of [[Infants]] and [[Children]] with [[Congenital Syphilis]]'''
|-
!'''[[Congenital Syphilis]] in [[infants]] and [[children]]'''
|
*[[Infants]] and [[children]] aged ≥1 month who are identified as having reactive [[serologic]] tests for [[syphilis]] should be examined thoroughly and have [[maternal]] [[serology]] and records reviewed to assess whether they have [[congenital]] or acquired [[syphilis]].
*Any [[infant]] or [[child]] at risk for [[congenital syphilis]] should receive a full evaluation and testing for [[HIV]] infection
'''Recommended Evaluation'''
*[[CSF]] analysis for [[VDRL]], cell count, and protein
*CBC, differential, and [[platelet count]]
*Other tests as clinically indicated (e.g., long-bone radiographs, [[chest radiograph]], [[liver function]] tests, [[abdominal ultrasound]], ophthalmologic examination, neuroimaging, and auditory brain-stem response)
 
'''Preferred regimen:''' [[Aqueous crystalline penicillin G]] 50,000 U/kg q4–6h for 10 days<br>
*If the [[infant]] or [[child]] has no clinical manifestations of [[congenital syphilis]] and the evaluation (including the [[CSF]] examination) is normal, treatment with up to 3 weekly doses of [[benzathine penicillin G]], 50,000 U/kg IM can be considered. A single dose of [[benzathine penicillin G]] 50,000 units/kg IM up to the adult dose of 2.4 million units in a single dose can be considered after the 10-day course of IV aqueous [[penicillin]] to provide more comparable duration of treatment in those who have no clinical manifestations and normal [[CSF]]. All of the above treatment regimens also would be adequate for children who might have other [[treponemal]] infections.
|-
!'''Follow Up'''
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*Careful follow-up examinations and [[serologic testing]] (i.e., a nontreponemal test) of [[infants]] and [[children]] treated for [[congenital syphilis]] after the [[neonatal]] period (30 days of age) should be performed every 3 months until the test becomes nonreactive or the titer has decreased fourfold.
*If the titers increase at any point for more than 2 weeks or do not decrease fourfold after 12–18 months, the infant or child should be evaluated (e.g., through [[CSF]] examination), treated with a 10-day course of parenteral [[penicillin G]], and managed in consultation with an expert.
* [[Treponemal]] tests should not be used to evaluate treatment response, because the results are qualitative and persist after treatment; further, passive transfer of maternal [[IgG]] [[treponemal]] [[antibody]] might persist for at least 15 months after delivery.
*Infants or children whose initial [[CSF]] evaluations are abnormal should undergo a repeat [[lumbar puncture]] approximately every 6 months until the results are normal. After 2 years of follow-up, a reactive [[CSF]] [[VDRL]] test or abnormal [[CSF]] indices that persists and cannot be attributed to other ongoing illness requires retreatment for possible [[neurosyphilis]] and should be managed in consultation with an expert.
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!'''Penicillin Allergy'''
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*[[Infants]] and children who require treatment for [[congenital syphilis]] but who have a history of [[penicillin]] allergy or develop an allergic reaction presumed secondary to [[penicillin]] should be desensitized and treated with [[penicillin]]
|}
 
==Prevention==
 
===Primary Prevention===
[[Primary prevention]] of [[syphilis]] in women of reproductive age and  men who have sex with women and prevention of mother to infant transmission in infected individuals plays a important role in decreasing incidence of [[congenital syphilis]].Effective measures for the [[primary prevention]] of [[congenital syphilis]] include reducing the risk of mother having syphilis infection and also screening during the antenatal period:<ref name="pmid19805553">{{cite journal |author=Stamm LV |title=Global challenge of atibiotic-resistant Treponema pallidum |journal=[[Antimicrobial Agents and Chemotherapy]] |volume=54 |issue=2 |pages=583–9 |year=2010 |month=February |pmid=19805553 |pmc=2812177 |doi=10.1128/AAC.01095-09 |url=http://aac.asm.org/cgi/pmidlookup?view=long&pmid=19805553 |accessdate=2012-02-21}}</ref><ref name="pmid24135571">{{cite journal |vauthors=Cameron CE, Lukehart SA |title=Current status of syphilis vaccine development: need, challenges, prospects |journal=Vaccine |volume=32 |issue=14 |pages=1602–9 |year=2014 |pmid=24135571 |pmc=3951677 |doi=10.1016/j.vaccine.2013.09.053 |url=}}</ref><ref name=CDCsyphilis>http://www.cdc.gov/std/tg2015/syphilis.htm Accessed on September 27, 2016</ref><ref name="pmid27081586">{{cite journal| author=Lago EG| title=Current Perspectives on Prevention of Mother-to-Child Transmission of Syphilis. | journal=Cureus | year= 2016 | volume= 8 | issue= 3 | pages= e525 | pmid=27081586 | doi=10.7759/cureus.525 | pmc=4829408 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27081586  }} </ref>
*Routine screening in [[pregnant]] females, individuals with high risk behaviours, and those residing in highly prevalent areas.
*[[Abstinence]] from intimate physical contact with an infected person.
*Consistent use of latex condoms.
*Limiting no of sexual partners.
*Avoid sharing sex toys.
*Practising safe sex.
 
===Secondary Prevention===
*Regular follow up of [[infants]] with [[congenital syphilis]] to examine for the re-appearance of [[signs]] and [[symptoms]] of [[syphilis]] after recommended treatment has shown to improve outcomes.<ref name="pmid28146163">{{cite journal| author=Feliz MC, Prizybicien AR, Rossoni AM, Tahnus T, Pereira AM, Rodrigues C| title=Adherence to the follow-up of the newborn exposed to syphilis and factors associated with loss to follow-up. | journal=Rev Bras Epidemiol | year= 2016 | volume= 19 | issue= 4 | pages= 727-739 | pmid=28146163 | doi=10.1590/1980-5497201600040004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28146163  }} </ref><ref name="pmid26474815">{{cite journal| author=Wallace HE, Broomhall HM, Isitt CE, Miall LS, Wilson JD| title=Serological follow-up of infants born to mothers with positive syphilis serology - real-world experiences. | journal=Int J STD AIDS | year= 2016 | volume= 27 | issue= 13 | pages= 1213-1217 | pmid=26474815 | doi=10.1177/0956462415612394 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26474815  }} </ref><ref name="pmid27622443">{{cite journal| author=Vallejo C, Cifuentes Y| title=Characterization and six-month follow-up on a cohort of newborns with congenital syphilis. | journal=Biomedica | year= 2016 | volume= 36 | issue= 1 | pages= 101-8 | pmid=27622443 | doi=10.7705/biomedica.v36i1.2661 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27622443  }} </ref>


==References==
{{reflist|2}}


{{WH}}
==Differential Diagnosis==
{{WS}}

Revision as of 18:58, 6 March 2017


Overview

Causes

Classification

Differential Diagnosis