
Congenital syphilis is
syphilis
Syphilis () is a sexually transmitted infection caused by the bacterium ''Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms depend on the stage it presents: primary, secondary, latent syphilis, latent or tertiary. The prim ...
that occurs when a mother with untreated syphilis passes the infection to her baby during
pregnancy
Pregnancy is the time during which one or more offspring gestation, gestates inside a woman's uterus. A multiple birth, multiple pregnancy involves more than one offspring, such as with twins.
Conception (biology), Conception usually occurs ...
or at
birth
Birth is the act or process of bearing or bringing forth offspring, also referred to in technical contexts as parturition. In mammals, the process is initiated by hormones which cause the muscular walls of the uterus to contract, expelling the f ...
.
It may present in the
fetus
A fetus or foetus (; : fetuses, foetuses, rarely feti or foeti) is the unborn offspring of a viviparous animal that develops from an embryo. Following the embryonic development, embryonic stage, the fetal stage of development takes place. Pren ...
,
infant
In common terminology, a baby is the very young offspring of adult human beings, while infant (from the Latin word ''infans'', meaning 'baby' or 'child') is a formal or specialised synonym. The terms may also be used to refer to juveniles of ...
, or later.
Clinical features vary and differ between early onset, that is presentation before 2-years of age, and late onset, presentation after age 2-years.
[ Infection in the unborn baby may present as poor growth, non-immune hydrops leading to ]premature birth
Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is betwee ...
or loss of the baby, or no signs.[ Affected newborns mostly initially have no clinical signs.][ They may be ]small
Small means of insignificant size
Size in general is the Magnitude (mathematics), magnitude or dimensions of a thing. More specifically, ''geometrical size'' (or ''spatial size'') can refer to three geometrical measures: length, area, or ...
and irritable.[ Characteristic features include a rash, ]fever
Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...
, large liver and spleen, a runny and congested nose, and inflammation around bone or cartilage
Cartilage is a resilient and smooth type of connective tissue. Semi-transparent and non-porous, it is usually covered by a tough and fibrous membrane called perichondrium. In tetrapods, it covers and protects the ends of long bones at the joints ...
.[ There may be ]jaundice
Jaundice, also known as icterus, is a yellowish or, less frequently, greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving ...
, large glands, pneumonia
Pneumonia is an Inflammation, inflammatory condition of the lung primarily affecting the small air sacs known as Pulmonary alveolus, alveoli. Symptoms typically include some combination of Cough#Classification, productive or dry cough, ches ...
( pneumonia alba), meningitis
Meningitis is acute or chronic inflammation of the protective membranes covering the brain and spinal cord, collectively called the meninges. The most common symptoms are fever, intense headache, vomiting and neck stiffness and occasion ...
, warty bumps on genitals, deafness or blindness. Untreated babies that survive the early phase may develop skeletal deformities including deformity of the nose
A nose is a sensory organ and respiratory structure in vertebrates. It consists of a nasal cavity inside the head, and an external nose on the face. The external nose houses the nostrils, or nares, a pair of tubes providing airflow through the ...
, lower legs, forehead
In human anatomy, the forehead is an area of the head bounded by three features, two of the skull and one of the scalp. The top of the forehead is marked by the hairline, the edge of the area where hair on the scalp grows. The bottom of the fo ...
, collar bone
The clavicle, collarbone, or keybone is a slender, S-shaped long bone approximately long that serves as a strut between the shoulder blade and the sternum (breastbone). There are two clavicles, one on each side of the body. The clavicle is the ...
, jaw
The jaws are a pair of opposable articulated structures at the entrance of the mouth, typically used for grasping and manipulating food. The term ''jaws'' is also broadly applied to the whole of the structures constituting the vault of the mouth ...
, and cheek bone.[ There may be a perforated or high arched ]palate
The palate () is the roof of the mouth in humans and other mammals. It separates the oral cavity from the nasal cavity.
A similar structure is found in crocodilians, but in most other tetrapods, the oral and nasal cavities are not truly sep ...
, and recurrent joint disease.[ Other late signs include linear perioral tears, ]intellectual disability
Intellectual disability (ID), also known as general learning disability (in the United Kingdom), and formerly mental retardation (in the United States), Rosa's Law, Pub. L. 111-256124 Stat. 2643(2010).Archive is a generalized neurodevelopmental ...
, hydrocephalus
Hydrocephalus is a condition in which cerebrospinal fluid (CSF) builds up within the brain, which can cause pressure to increase in the skull. Symptoms may vary according to age. Headaches and double vision are common. Elderly adults with n ...
, and juvenile general paresis.[ ]Seizures
A seizure is a sudden, brief disruption of brain activity caused by abnormal, excessive, or synchronous neuronal firing. Depending on the regions of the brain involved, seizures can lead to changes in movement, sensation, behavior, awareness, o ...
and cranial nerve palsies
Cranial nerve disease is an impaired functioning of one of the twelve cranial nerves. Although it could theoretically be considered a mononeuropathy, it is not considered as such under MeSH.
It is possible for a disorder of more than one cran ...
may first occur in both early and late phases.[ Eighth nerve palsy, interstitial keratitis and small notched teeth may appear individually or together; known as ]Hutchinson's triad
Hutchinson triad is a triad of signs that may be seen in late congenital syphilis, including: interstitial keratitis, malformed teeth ( Hutchinson incisors and mulberry molars), and eighth nerve deafness.
Late congenital syphilis typically man ...
.[
It is caused by the ]bacterium
Bacteria (; : bacterium) are ubiquitous, mostly free-living organisms often consisting of one biological cell. They constitute a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria were among the ...
''Treponema pallidum
''Treponema pallidum'', formerly known as ''Spirochaeta pallida'', is a Microaerophile, microaerophilic, Gram-negative bacteria, gram-negative, spirochaete bacterium with subspecies that cause the diseases syphilis, bejel (also known as endemic ...
'' subspecies
In Taxonomy (biology), biological classification, subspecies (: subspecies) is a rank below species, used for populations that live in different areas and vary in size, shape, or other physical characteristics (Morphology (biology), morpholog ...
''pallidum'' when it infects the baby after crossing the placenta or from contact with a syphilitic sore at birth.[ It is not transmitted during ]breastfeeding
Breastfeeding, also known as nursing, is the process where breast milk is fed to a child. Infants may suck the milk directly from the breast, or milk may be extracted with a Breast pump, pump and then fed to the infant. The World Health Orga ...
unless there is an open sore on the mother's breast
The breasts are two prominences located on the upper ventral region of the torso among humans and other primates. Both sexes develop breasts from the same embryology, embryological tissues. The relative size and development of the breasts is ...
.[ The unborn baby can become infected at any time during the pregnancy.][ Most cases occur due to inadequate antenatal screening and treatment during pregnancy.] The baby is highly infectious if the rash and snuffles are present.[ The disease may be suspected from tests on the mother; blood tests and ]ultrasound
Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...
. Tests on the baby may include blood tests, CSF analysis and medical imaging
Medical imaging is the technique and process of imaging the interior of a body for clinical analysis and medical intervention, as well as visual representation of the function of some organs or tissues (physiology). Medical imaging seeks to revea ...
. Findings may reveal anemia
Anemia (also spelt anaemia in British English) is a blood disorder in which the blood has a reduced ability to carry oxygen. This can be due to a lower than normal number of red blood cells, a reduction in the amount of hemoglobin availabl ...
and low platelets.[ Other findings may include low sugars, ]proteinuria
Proteinuria is the presence of excess proteins in the urine. In healthy persons, urine contains very little protein, less than 150 mg/day; an excess is suggestive of illness. Excess protein in the urine often causes the urine to become fo ...
and hypopituitarism.[ The ]placenta
The placenta (: placentas or placentae) is a temporary embryonic and later fetal organ that begins developing from the blastocyst shortly after implantation. It plays critical roles in facilitating nutrient, gas, and waste exchange between ...
may appear large and pale.[ Other investigations include testing for HIV.][
Prevention is by ]safe sex
Safe sex is sexual activity using methods or contraceptive devices (such as condoms) to reduce the risk of transmitting or acquiring sexually transmitted infections (STIs), especially HIV. "Safe sex" is also sometimes referred to as safer ...
to prevent syphilis in the mother, and early screening and treatment of syphilis in pregnancy.[ One intramuscular injection of benzathine penicillin G administered to a pregnant woman early in the illness can prevent congenital syphilis in her baby.] Treatment of suspected congenital syphilis is with penicillin
Penicillins (P, PCN or PEN) are a group of beta-lactam antibiotic, β-lactam antibiotics originally obtained from ''Penicillium'' Mold (fungus), moulds, principally ''Penicillium chrysogenum, P. chrysogenum'' and ''Penicillium rubens, P. ru ...
by injection; benzylpenicillin
Benzylpenicillin, also known as penicillin G (PenG) or BENPEN, is an antibiotic used to treat a number of bacterial infections. This includes pneumonia, strep throat, syphilis, necrotizing enterocolitis, diphtheria, gas gangrene, leptospiro ...
into vein, or procaine benzylpenicillin
Procaine benzylpenicillin also known as penicillin G procaine, is an antibiotic useful for the treatment of a number of bacterial infections. Specifically it is used for syphilis, anthrax, mouth infections, pneumonia, diphtheria, cellulitis, a ...
into muscle.[ During times of penicillin unavailability, ]ceftriaxone
Ceftriaxone, sold under the brand name Rocephin, is a third-generation cephalosporin antibiotic used for the treatment of a number of bacterial infections. These include middle ear infections, endocarditis, meningitis, pneumonia, bone and joi ...
may be an alternative.[ Where there is penicillin allergy, antimicrobial desensitisation is an option.]
Syphilis affects around one million pregnancies a year. In 2016, there were around 473 cases of congenital syphilis per 100,000 live births and 204,000 deaths from the disease worldwide. Of the 660,000 congenital syphilis cases reported in 2016, 143,000 resulted in deaths of unborn babies, 61,000 deaths of newborn babies, 41,000 low birth weights or preterm births, and 109,000 young children diagnosed with congenital syphilis. Around 75% were from the WHO's African and Eastern Mediterranean regions.[ Serological tests for syphilis were introduced in 1906, and it was later shown that transmission occurred from the mother.]
Signs and symptoms
Untreated early syphilis infections results in a high risk of poor pregnancy outcomes, including saddle nose, lower extremity abnormalities, miscarriage
Miscarriage, also known in medical terms as a spontaneous abortion, is an end to pregnancy resulting in the loss and expulsion of an embryo or fetus from the womb before it can fetal viability, survive independently. Miscarriage before 6 weeks ...
s, premature birth
Preterm birth, also known as premature birth, is the birth of a baby at fewer than 37 weeks gestational age, as opposed to full-term delivery at approximately 40 weeks. Extreme preterm is less than 28 weeks, very early preterm birth is betwee ...
s, stillbirth
Stillbirth is typically defined as fetus, fetal death at or after 20 or 28 weeks of pregnancy, depending on the source. It results in a baby born without vital signs, signs of life. A stillbirth can often result in the feeling of guilt (emotio ...
s, or death in newborns
In common terminology, a baby is the very young offspring of adult human beings, while infant (from the Latin word ''infans'', meaning 'baby' or 'child') is a formal or specialised synonym. The terms may also be used to refer to juveniles of ...
. Some infants with congenital syphilis have symptoms at birth, but many develop symptoms later. Symptoms may include rash, fever, large liver and spleen, and skeletal abnormalities. Newborns will typically not develop a primary syphilitic chancre but may present with signs of secondary syphilis (i.e. generalized body rash). Often these babies will develop syphilitic rhinitis
Rhinitis, also known as coryza, is irritation and inflammation of the mucous membrane inside the nose. Common symptoms are a stuffy nose, runny nose, sneezing, and post-nasal drip.
The inflammation is caused by viruses, bacteria, irritant ...
("snuffles"), the mucus from which is laden with the ''Treponema pallidum
''Treponema pallidum'', formerly known as ''Spirochaeta pallida'', is a Microaerophile, microaerophilic, Gram-negative bacteria, gram-negative, spirochaete bacterium with subspecies that cause the diseases syphilis, bejel (also known as endemic ...
'' bacterium, and therefore highly infectious. If a baby with congenital syphilis is not treated early, damage to the bones, teeth, eyes, ears, and brain can occur.
Neurosyphilis in newborns may present as cranial nerve palsies
Cranial nerve disease is an impaired functioning of one of the twelve cranial nerves. Although it could theoretically be considered a mononeuropathy, it is not considered as such under MeSH.
It is possible for a disorder of more than one cran ...
, cerebral infarcts (strokes), seizures
A seizure is a sudden, brief disruption of brain activity caused by abnormal, excessive, or synchronous neuronal firing. Depending on the regions of the brain involved, seizures can lead to changes in movement, sensation, behavior, awareness, o ...
or eye abnormalities.
Many newborns with congenital syphilis, 55% by some estimates, do not exhibit any symptoms initially, with signs and symptoms developing days to months later.
Early
Newborns may be asymptomatic and are only identified on routine prenatal screening. If not identified and treated, these newborns develop poor feeding and runny nose. By definition, early congenital syphilis occurs in children between 0 and 2 years old.
Late
Congenital syphilis that is diagnosed after 2 years of age, either because it was not diagnosed earlier or because it was incompletely treated, is classified as late congenital syphilis. The signs of late congenital syphilis tend to reflect early damage to developing tissues that does not become apparent until years later, such as Hutchinson's triad
Hutchinson triad is a triad of signs that may be seen in late congenital syphilis, including: interstitial keratitis, malformed teeth ( Hutchinson incisors and mulberry molars), and eighth nerve deafness.
Late congenital syphilis typically man ...
of Hutchinson's teeth (notched incisors), keratitis and deafness.
Symptoms include:
* Blunted upper incisor teeth known as Hutchinson's teeth
Hutchinson's teeth is a sign of congenital syphilis. Affected people have Human tooth, teeth that are smaller and more widely spaced than normal and which have notches on their biting surfaces.
It is named for Jonathan Hutchinson, Sir Jonathan ...
, or mulberry molars
* Deafness
Deafness has varying definitions in cultural and medical contexts. In medical contexts, the meaning of deafness is hearing loss that precludes a person from understanding spoken language, an audiological condition. In this context it is writte ...
from auditory nerve disease
* Frontal bossing (prominence of the brow ridge)
* Hard palate
The palate () is the roof of the mouth in humans and other mammals. It separates the oral cavity from the nasal cavity.
A similar structure is found in crocodilians, but in most other tetrapods, the oral and nasal cavities are not truly sep ...
defect
* Inflammation of the cornea known as interstitial keratitis
Keratitis is a condition in which the human eye, eye's cornea, the clear dome on the front surface of the eye, becomes inflammation, inflamed. The condition is often marked by moderate to intense pain and usually involves any of the following sy ...
* Protruding mandible
* Saber shin
Saber shin is a malformation of the tibia. It presents as a sharp anterior bowing, or convexity, of the tibia.
__TOC__
Causes
Periosteal reaction along the shaft of the tibia. It can result from congenital syphilis, yaws, Paget's disease of bo ...
s
* Saddle nose (collapse of the bony part of nose)
* Short maxillae
* Swollen knees
Treatment (with penicillin) before the development of late symptoms is essential.
Clinical signs include:
* Abnormal bone x-rays
An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...
* Anemia
Anemia (also spelt anaemia in British English) is a blood disorder in which the blood has a reduced ability to carry oxygen. This can be due to a lower than normal number of red blood cells, a reduction in the amount of hemoglobin availabl ...
* Cerebral palsy
Cerebral palsy (CP) is a group of movement disorders that appear in early childhood. Signs and symptoms vary among people and over time, but include poor coordination, spasticity, stiff muscles, Paresis, weak muscles, and tremors. There may b ...
* Du Bois sign, shortening of the little finger
* Enlarged liver[
* ]Enlarged spleen
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulatin ...
[
* Frontal bossing]
* Sensorineural hearing loss
Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spo ...
* Higouménakis' sign, enlargement of the sternal end of clavicle in late congenital syphilis, mostly on the right-hand side
* Hutchinson's triad
Hutchinson triad is a triad of signs that may be seen in late congenital syphilis, including: interstitial keratitis, malformed teeth ( Hutchinson incisors and mulberry molars), and eighth nerve deafness.
Late congenital syphilis typically man ...
, a set of symptoms consisting of deafness
Deafness has varying definitions in cultural and medical contexts. In medical contexts, the meaning of deafness is hearing loss that precludes a person from understanding spoken language, an audiological condition. In this context it is writte ...
, Hutchinson's teeth
Hutchinson's teeth is a sign of congenital syphilis. Affected people have Human tooth, teeth that are smaller and more widely spaced than normal and which have notches on their biting surfaces.
It is named for Jonathan Hutchinson, Sir Jonathan ...
(centrally notched, widely spaced peg-shaped upper central incisors
Incisors (from Latin ''incidere'', "to cut") are the front teeth present in most mammals. They are located in the premaxilla above and on the mandible below. Humans have a total of eight (two on each side, top and bottom). Opossums have 18, wher ...
), and interstitial keratitis
Keratitis is a condition in which the human eye, eye's cornea, the clear dome on the front surface of the eye, becomes inflammation, inflamed. The condition is often marked by moderate to intense pain and usually involves any of the following sy ...
(IK), an inflammation of the cornea which can lead to corneal scarring and potential blindness
* Hydrocephalus
Hydrocephalus is a condition in which cerebrospinal fluid (CSF) builds up within the brain, which can cause pressure to increase in the skull. Symptoms may vary according to age. Headaches and double vision are common. Elderly adults with n ...
* Jaundice
Jaundice, also known as icterus, is a yellowish or, less frequently, greenish pigmentation of the skin and sclera due to high bilirubin levels. Jaundice in adults is typically a sign indicating the presence of underlying diseases involving ...
* Lymph node enlargement
* Mulberry molars (permanent first molars with multiple poorly developed cusps)
* Musculoskeletal deformities
* Petechiae
A petechia (; : petechiae) is a small red or purple spot ( 1 cm in diameter) and purpura (3 to 10 mm in diameter). The term is typically used in the plural (petechiae), since a single petechia is seldom noticed or significant.
Causes Physical ...
* Poorly developed maxillae
* Pseudoparalysis
* Rhagades, linear scars at the angles of the mouth and nose result from bacterial infection of skin lesions
* Snuffles, aka "syphilitic rhinitis", which appears similar to the rhinitis of the common cold, except it is more severe, lasts longer, often involves bloody rhinorrhea, and is often associated with laryngitis
* Sabre shins, malformation of the tibia
* Skin rash
A rash is a change of the skin that affects its color, appearance, or texture.
A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cracked ...
or other skin changes such as rhagades
Death from congenital syphilis is usually due to bleeding into the lungs.
Transmission
Syphilis may be transmitted from mother to the fetus during any stage of pregnancy. It is most commonly transmitted via cross placental transfer of ''Treponema pallidum'' bacteria from mother to the fetus during pregnancy with transmission via exposure to genital lesions during childbirth being less common. The highest rate of transmission occurs in mothers with early syphilis (infection present for less than 1 year), which is responsible for 50-70% of infections, with syphilis being present for more than 1 year thought to be responsible for about 15% of transmission. It is not transmitted during breastfeeding
Breastfeeding, also known as nursing, is the process where breast milk is fed to a child. Infants may suck the milk directly from the breast, or milk may be extracted with a Breast pump, pump and then fed to the infant. The World Health Orga ...
unless there is an open sore on the mother's breast
The breasts are two prominences located on the upper ventral region of the torso among humans and other primates. Both sexes develop breasts from the same embryology, embryological tissues. The relative size and development of the breasts is ...
.[
]
Diagnosis
Direct observation of ''Treponema pallidum'' from one of the lesions in the mother or infant is diagnostic and can be carried out using dark field microscopy
Dark-field microscopy, also called dark-ground microscopy, describes microscopy methods, in both light microscopy, light and electron microscopy, which exclude the unscattered beam from the image. Consequently, the field around the specimen (i.e ...
, direct fluorescent antibody testing or immunohistochemical staining
Immunohistochemistry is a form of immunostaining. It involves the process of selectively identifying antigens in cells and tissue, by exploiting the principle of antibodies binding specifically to antigens in biological tissues. Albert Hewett ...
, however these tests are not readily available in many settings. Serological testing is more commonly carried out on the mother and the infant to diagnose maternal and congenital syphilis. In the mother, a serologic diagnosis of syphilis is made using a nontreponemal test for syphilis such as the Venereal Disease Research Laboratory test ( VDRL) or Rapid plasma reagin
The rapid plasma reagin test (RPR test or RPR titer) is a type of rapid diagnostic test that looks for non-specific antibodies in the blood of the patient that may indicate an infection by syphilis or related non-venereal treponematoses. It is ...
(RPR) followed by a treponemal test, such as the ''Treponema pallidum'' particle agglutination assay (TP-PA) (the sequence of testing may be reversed with a treponemal test followed by a non-treponemal test in the reverse diagnostic sequence). Positivity of both tests indicates active syphilis or previous infection that was treated. Quantitative non-treponemal tests are monitored for disease activity and response to treatment, with RPR is expected to decrease by a factor of four compared to pre-treatment levels after successful treatment of syphilis in the mother. Failure of non-treponemal titers to decrease after treatment may indicate treatment failure or re-infection. A confirmed cure in the mother does not exclude the possibility of congenital syphilis as transmission to the fetus may have occurred prior to maternal cure.
Diagnosis of congenital syphilis in the fetus is based on a combination of laboratory, imaging and physical exam findings. Ultrasound findings associated with congenital syphilis intrauterine infection (which are seen after 18 weeks gestation) include fetal hepatomegaly
Hepatomegaly is enlargement of the liver. It is a non-specific sign (medicine), medical sign, having many causes, which can broadly be broken down into infection, hepatic tumours, and metabolic disorder. Often, hepatomegaly presents as an abdomin ...
(enlarged liver)(seen in greater than 80% of cases), anemia (as measured by the peak systolic velocity of the middle cerebral artery)(33%), placentomegaly(an enlarged placenta)(27%), polyhydramnios
Polyhydramnios is a medical condition describing an excess of amniotic fluid in the amniotic sac. It is seen in about 1% of pregnancies. It is typically diagnosed when the amniotic fluid index (AFI) is greater than 24 cm.
There are two clini ...
(an excess of amniotic fluid in the amniotic sac)(12%) and hydrops fetalis
Hydrops fetalis or hydrops foetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in ...
(edema in the fetus)(10%). The absence of these ultrasound findings does not rule out congenital syphilis in the fetus. These ultrasound abnormalities usually resolve several weeks after successful treatment of syphilis in the mother.
Immunohistochemical staining or nucleic acid amplification of the amniotic fluid may also aid in the diagnosis. Diagnosis of syphilis in the neonate may be challenging as maternal treponemal antibodies (as non-treponemal titers) can cross the placenta and persist in the infant for many months after birth in the absence of neonatal syphilis, complicating the diagnosis. The passively transferred non-treponemal titers should be cleared from the infant within 15 months of birth (with most titers being cleared by 6 months), and persistently elevated titers 6 months after birth should prompt investigation into neonatal syphilis including CSF analysis for neurosyphilis. Pleocytosis, raised CSF protein level and positive CSF serology suggest neurosyphilis. CSF VDRL is 50-90% specific for neurosyphilis. 60% of newborns with congenital syphilis also have neurosyphilis. Non-treponemal titers should be monitored in the newborns every 2-3 months to ensure an adequate response to treatment.
Treatment
If a pregnant mother is identified as being infected with syphilis, treatment can effectively prevent congenital syphilis from developing in the fetus, especially if she is treated before the sixteenth week of pregnancy or at least 30 days prior to delivery.[ Mothers with primary syphilis can be treated with a single dose of intramuscularly injected penicillin, whereas late-latent, secondary syphilis, or disease of an unknown duration is treated with once weekly penicillin injections for three weeks.] The mother's partner should also be evaluated and treated.
The fetus is at greatest risk of contracting syphilis when the mother is in the early stages of infection, but the disease can be passed at any point during pregnancy, even during delivery. An affected child can be treated using antibiotics much like an adult; however, any developmental symptoms are likely to be permanent.
The greater the duration between the infection
An infection is the invasion of tissue (biology), tissues by pathogens, their multiplication, and the reaction of host (biology), host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmis ...
of the mother and conception, the better the outcome for the infant
In common terminology, a baby is the very young offspring of adult human beings, while infant (from the Latin word ''infans'', meaning 'baby' or 'child') is a formal or specialised synonym. The terms may also be used to refer to juveniles of ...
including less chance of stillbirth or developing congenital syphilis.
The Centers for Disease Control and Prevention
The Centers for Disease Control and Prevention (CDC) is the National public health institutes, national public health agency of the United States. It is a Federal agencies of the United States, United States federal agency under the United S ...
recommends treating symptomatic or babies born to an infected mother with unknown treatment status with procaine penicillin G, 50,000 U/kg dose IM a day in a single dose for 10 days. Treatment for these babies can vary on a case-by-case basis. Treatment cannot reverse any deformities, brain, or permanent tissue damage that has already occurred.
A Cochrane review
Cochrane is a British international charitable organisation formed to synthesize medical research findings to facilitate evidence-based choices about health interventions involving health professionals, patients and policy makers. It includes ...
found that antibiotics may be effective for serological cure but in general the evidence around the effectiveness of antibiotics for congenital syphilis is uncertain due to the poor methodological quality of the small number of trials that have been conducted.
Up to 40% of pregnant women treated for congenital syphilis will develop a Jarisch–Herxheimer reaction, which is a temporary reaction that usually occurs within a few hours of starting penicillin and resolves by 24 hours. The reaction is characterized by cramping, fever, muscle aches and a rash. Treatment is supportive and fetal heart rate monitoring is recommended.
Epidemiology
Syphilis affects around one million pregnancies a year.[ In 2016, there were around 473 cases of congenital syphilis per 100,000 live births and 204,000 deaths from the disease worldwide.][ Of the 660,000 congenital syphilis cases reported in 2016, 143,000 resulted in deaths of unborn babies, 61,000 deaths of newborn babies, 41,000 low birth weights or preterm births, and 109,000 young children diagnosed with congenital syphilis.][ Around 75% were from the WHO's African and Eastern Mediterranean regions.][
Cases of congenital syphilis in the United States have been rising since the early 2010s. The ]Centers for Disease Control and Prevention
The Centers for Disease Control and Prevention (CDC) is the National public health institutes, national public health agency of the United States. It is a Federal agencies of the United States, United States federal agency under the United S ...
(CDC) reported 918 cases for 2017, which is more than twice the yearly incidence of the preceding four years. The incidence in the United States has increased by 754% from 2012 to 2021 with a higher incidence seen in those with a lower socioeconomic status, as well as Black people, Native Americans and Native Hawaiians. Reports in 2023 show a rise of more than 900 percent in Mississippi
Mississippi ( ) is a U.S. state, state in the Southeastern United States, Southeastern and Deep South regions of the United States. It borders Tennessee to the north, Alabama to the east, the Gulf of Mexico to the south, Louisiana to the s ...
over the preceding five years.
History
Congenital syphilis was first described in Europe during the fifteenth century by the Spanish physician . Nineteenth century physicians held the belief that congenital syphilis was contracted from contaminated semen at time of conception.[ Serological tests for syphilis were introduced in 1906, and it was later shown that transmission occurred from the mother.][
]
References
External links
{{Diseases of maternal transmission
Infections with a predominantly sexual mode of transmission
Infections specific to the perinatal period
Syphilis
Disorders causing seizures