About two thirds of all children suffering from CS are born without any symptoms. The disease is often detected during routine screenings.

The first symptoms may appear weeks, even a few months after birth. They include skin lesions such as blisters and copper-colored rash on palms and soles, papules in face and lower abdomen and petechiae. Children often develop a purulent rhinitis and parents frequently report their children to snuffle. Of note, nasal discharge and skin lesions are highly contagious. A clinical examination may further reveal enlargement of lymph nodes, liver and spleen. Hepatomegaly is indeed the most common finding in CS and is usually associated with altered results of serum liver enzymes. More severe symptoms such as pneumonia, hemolytic anemia, thrombocytopenia and less commonly leukocytosis may be observed. Neurological symptoms may be present and consist in hydrocephalus, meningitis, seizures and consecutive mental retardation. Alterations in cerebrospinal fluid (CSF) composition may be found. Physical development may also be retarded.

When children affected by CS reach the age of half a year, they may develop osteochondritis. It may lead to pseudoparalysis and is usually not detectable in younger children [10]. This condition mainly affects the long bones of the limbs as well as the ribs and therefore strongly impairs the child's movements. Bone lesions matching with chondroepiphysitis, ostitis or periostitis are detectable in radiographic images and are of great diagnostic value because they can indeed be found in children who show hardly any additional signs of CS [11].

Clinical symptoms are different in children developing late CS, that is who manifest symptoms after reaching the age of two. Ulcerative lesions in mucosa of nose and hard palate are common, as are periostal lesions resulting in leg and head deformations (saber shins, skull bossing). The nervous system may be affected, but symptoms are rare. Tabes dorsalis and paresis as well as progressive loss of hearing due to damage to the eighth cranial nerve may possibly be observed. The eyes may suffer from interstitial keratitis, consecutive corneal scarring, but also from optic atrophy leading to blindness. Tooth deformities such as Hutchinson incisors and mulberry molars are as characteristic for late CS as perioral fissures.

• Generalized Lymphadenopathy

  These neonates also display significant changes in bone morphology and frequently hepatosplenomegaly along with generalized lymphadenopathy. [pathwaymedicine.org]

  The various signs associated with early CS are listed below: General symptoms The general symptoms include: Fever Failure to thrive Generalized lymphadenopathy Intrauterine growth retardation Skin problems The symptoms associated with skin include: Condyloma [hxbenefit.com]

  Generalized lymphadenopathy and hepatosplenomegaly often occur. The infant may fail to thrive and have a characteristic mucopurulent or blood-stained nasal discharge causing snuffles. [msdmanuals.com]

  These include low-grade fever, malaise, and generalized lymphadenopathy. Some disability may result from involvement of palms and soles. Periosteitis and rarefaction are more extreme in children. [drmhijazy.com]

• Splenomegaly

  Compared with term neonates with CS, preterm ones were more likely to have characteristic skin rash (36.2 vs. 9.7%, p < 0.001), hepatomegaly (51.7 vs. 25%, p = 0.02), splenomegaly (32.8 vs. 15.4%, p = 0.02), PRP titer ≥1:8 (96.6 vs. 70.8%, p < 0.001), [ncbi.nlm.nih.gov]

  Less common presentations include patchy alopecia, anterior uveitis, meningitis, cranial nerve palsies, hepatitis, splenomegaly, periostitis and glomerulonephritis. [patient.info]

• Fever

  We report an unusual case of recurrent fever, inflammatory knee pain, genu varum, persistent anemia, and high erythrocyte sedimentation rate in a 28-month-old boy as late manifestations of congenital syphilis (CS). [ncbi.nlm.nih.gov]

  This disease used to be called "break-bone" fever because it sometimes causes severe joint and muscle pain that feels like bones are breaking. Health experts have known about dengue fever for more than 200 years. [niaid.nih.gov]

  Babies who are untreated can suffer from: Deformities Developmental delays Seizures Rashes Fever Anemia Hepatosplenomegaly Jaundice The sores on the infected babies are generally infectious. [hxbenefit.com]

  If untreated, the infection may cause deafness, blindness, crippling, or death. congenital syphilis Congenital lues, fetal syphilis Neonatology Transplacental infection with Treponema pallidum Clinical Early–hepatomegaly, irritability, FTT, fever, perioral [medical-dictionary.thefreedictionary.com]

• Anemia

  The reason for anemia and thrombocytopenia in congenital syphilis is not clear. The authors suggest that hemophagocytosis may play role in pathogenesis of cytopenia, particularly thrombocytopenia in patients with congenital syphilis. [ncbi.nlm.nih.gov]

• Hutchinson's Triad

  One of the main aspects is observed with the triad of Hutchinson, characterised by the presence of interstitial keratitis, eighth nerve deafness and Hutchinson's teeth. [ncbi.nlm.nih.gov]

  One of the main aspects is observed with the triad of Hutchinson, characterised by the presence of interstitial keratitis, eighth nerve deafness and Hutchinson’s teeth. [casereports.bmj.com]

  Hutchinson's triad, a set of symptoms consisting of deafness, Hutchinson's teeth (centrally notched, widely spaced peg-shaped upper central incisors), and interstitial keratitis (IK), an inflammation of the cornea which can lead to corneal scarring and [en.wikipedia.org]

  Hutchinson’s triad (Hutchinson’s teeth, interstitial keratitis, and VIIIth nerve deafness) is considered pathognomonic of late congenital syphilis. [dartmouth.edu]

  The constellation of interstitial keratitis, cranial nerve VIII deafness, and Hutchinson teeth is called the Hutchinson triad. [aao.org]

• Weight Loss

  Secondary Syphilis About 2 to 10 weeks after the first sore appears, you may develop the following: A skin rash that causes small, reddish-brown sores Sores in your mouth, vagina, or anus Fever Swollen glands Weight loss Hair loss Headache Extreme tiredness [webmd.com]

  loss; and/or a sore throat and other flu-like symptoms, like fatigue. [americanpregnancy.org]

  Healing of these lesions lead to formation of radial scars known as " rhagades " Systemic Manifestations Old man look - due to constitutional symptoms as gastro-enteritis, pneumonia, weight loss and marasmus, which lead to wrinkled yellow brown skin. [drmhijazy.com]

• Pallor

  A less commonly described funduscopic variation is that of a bilateral secondary degeneration of the RPE, which may mimic retinitis pigmentosa with narrowing of the retinal and choroidal vessels, optic disc pallor with sharp margins, and morphologically [aao.org]

  Published Date 01 Jun 2018 Last Updated 21 Nov 2018 Apnoea is defined as no effective respiratory effort for ≥ 20 seconds or for > 10 seconds if associated with bradycardia (< 100 bpm), oxygen desaturation, cyanosis or pallor. [health.vic.gov.au]

  There was mild pallor and hepatosplenomegaly, with no icterus, lymphadenopathy, snuffles, feeding difficulty, rash, bulging anterior fontanel or cranial nerve palsy. [dx.doi.org]

• Saddle Nose

  Untreated early syphilis infections results in a high risk of poor pregnancy outcomes, including saddle nose, lower extremity abnormalities, miscarriages, premature births, stillbirths, or death in newborns. [en.wikipedia.org]

  History / PE : Early manifestations Hepatosplenomegaly Cutaneous lesions on palms/soles Jaundice/anemia Rhinorrhea Late/untreated manifestations Frontal bossing Hutchinson teeth Saddle nose Perioral fissures Diagnosis : VDRL FTA-ABS Treatment : Penicillin [medlibes.com]

  Congenital syphilis may feature severe early skin rashes, often occurring in the first 10 weeks of life, bone and cartilage defects, liver and kidney disturbances, damage to the corneas (interstitial keratitis), deafness, peg teeth, saddle-shaped nose [medical-dictionary.thefreedictionary.com]

  […] results from growth stimulation of the tibia with forward bowing (in rickets the bowing is lateral and not forward) Also may get dactylitis, especially of proximal phalanx of index finger DDX: Birth trauma, rickets, severe malnutrition Other changes Nose-saddle [learningradiology.com]

  Inflammation of bone's periosteum can lead to a number of morphological changes such as sinking in of the nose, resulting in a "Saddle Nose", or bowing of the tibia, resulting in "Saber Shins". [pathwaymedicine.org]

• Rhinitis

  Common clinical features of early congenital syphilis include hepatosplenomegaly, skeletal deformities, hematologic disturbances, and mucocutaneous features such as rhinitis and maculopapular rash. [ncbi.nlm.nih.gov]

  Often these babies will develop syphilitic rhinitis ("snuffles"), the mucus from which is laden with the T. pallidum bacterium, and therefore highly infectious. [en.wikipedia.org]

  although it appears similar to rhinitis of common cold, it is even more severe, lasts for a longer duration, frequently incorporates bloody rhinorrhea, and also frequently associated with laryngitis Congenital Syphilis Diagnosis A doctor conducts a [hxbenefit.com]

  Other symptoms include rhinitis, coryza, or snuffles. Snuffles usually occur in the first week of life, are very persistent and often bloody. [hon.ch]

  Many neonates possess a chronic rhinitis which is termed "snuffles" along with a skin rash similar to that observed in Secondary Syphilis. [pathwaymedicine.org]

• Rhinorrhea

  Untreated infants develop symptoms such as rhinorrhea, anemia, jaundice, cutaneous lesions, hepatosplenomegaly, and pseudoparalysis within weeks or months. [ncbi.nlm.nih.gov]

  History / PE : Early manifestations Hepatosplenomegaly Cutaneous lesions on palms/soles Jaundice/anemia Rhinorrhea Late/untreated manifestations Frontal bossing Hutchinson teeth Saddle nose Perioral fissures Diagnosis : VDRL FTA-ABS Treatment : Penicillin [medlibes.com]

  These newborns tend to develop rhinorrhea and poor feeding if they are not detected and treated in time. Early CS generally takes place in children who are aged between 0-2 years. [hxbenefit.com]

  […] petechiae. other skin rash. sabre shins. anemia. lymph node enlargement. jaundice. pseudoparalysis. 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 [en.wikipedia.org]

• Failure to Thrive

  Early signs are characteristic skin lesions, lymphadenopathy, hepatosplenomegaly, failure to thrive, blood-stained nasal discharge, perioral fissures, meningitis, choroiditis, hydrocephalus, seizures, intellectual disability, osteochondritis, and pseudoparalysis [msdmanuals.com]

  Signs and symptoms include irritability, fever, failure to thrive, saddle nose, cutaneous rash, and pneumonia. [fpnotebook.com]

  Rhinitis with highly infectious nasal discharge, nontender generalized adenopathy, alopecia, iritis, and failure to thrive occur less frequently. [dartmouth.edu]

  Congenital Syphilis Complications The following complications may develop in patients suffering from CS: Acidosis Deafness Blindness Pancreatitis Liver failure Hypoglycemia Severe anemia Failure to thrive Respiratory failure Thrombocytopenia Leukemoid [hxbenefit.com]

• Rectal Bleeding

  Intestinal obstruction and bleeding are uncommon complications of congenital syphilis (CS). A VDRL-positive infant developed incomplete intestinal obstruction and rectal bleeding. Despite conservative management, his symptoms continued. [ncbi.nlm.nih.gov]

• Hutchinson's Teeth

  Several reports in the literature have focused on oral manifestations of congenital syphilis, mainly about Hutchinson's teeth and dysplastic molars, which are more common. [ncbi.nlm.nih.gov]

  Later childhood signs of the infection include interstitial keratitis, deafness, and notches in the incisor teeth (called Hutchinson's teeth ). [medical-dictionary.thefreedictionary.com]

  Hutchinson's triad, a set of symptoms consisting of deafness, Hutchinson's teeth (centrally notched, widely spaced peg-shaped upper central incisors), and interstitial keratitis (IK), an inflammation of the cornea which can lead to corneal scarring and [en.wikipedia.org]

  Figure 2 Enamel hypoplasia of maxillary central incisors (Hutchinson’s teeth). Figure 3 The semilunar notch on the incisal edge of mandibular incisors (Hutchinson’s teeth). [casereports.bmj.com]

• Mulberry Molar

  Keywords treponematosis • pre-columbian • enamel hypoplasia • mulberry molar • congenital syphilis • hutchinson's incisor [schweizerbart.de]

  RESULTS: The observed morphology of the first molars corresponds to the typical aspect of mulberry molars, while that of the canines is characterised by hypomineralisation. Hypoplastic grooves were observed on the incisal edges of all incisors. [ncbi.nlm.nih.gov]

• Notched Incisors

  Hutchinson's triad, a set of symptoms consisting of deafness, Hutchinson's teeth (centrally notched, widely spaced peg-shaped upper central incisors), and interstitial keratitis (IK), an inflammation of the cornea which can lead to corneal scarring and [en.wikipedia.org]

  Syphilitic vasculitis can damage developing tooth buds, leading to peg-shaped, notched incisors (Hutchinson teeth) and multicusped first molars (mulberry molars). Deciduous teeth are not affected. [clinicaladvisor.com]

• Hepatosplenomegaly

  Common clinical features of early congenital syphilis include hepatosplenomegaly, skeletal deformities, hematologic disturbances, and mucocutaneous features such as rhinitis and maculopapular rash. [ncbi.nlm.nih.gov]

• Jaundice

  Untreated infants develop symptoms such as rhinorrhea, anemia, jaundice, cutaneous lesions, hepatosplenomegaly, and pseudoparalysis within weeks or months. [ncbi.nlm.nih.gov]

  Babies exposed in utero can have deformities, delays in development, or seizures along with many other problems such as rash, fever, an enlarged liver and spleen, anemia, and jaundice. [en.wikipedia.org]

  History / PE : Early manifestations Hepatosplenomegaly Cutaneous lesions on palms/soles Jaundice/anemia Rhinorrhea Late/untreated manifestations Frontal bossing Hutchinson teeth Saddle nose Perioral fissures Diagnosis : VDRL FTA-ABS Treatment : Penicillin [medlibes.com]

  The fetal stigmata seen before the age of 2 years include eruptions characteristic of secondary syphilis, such as maculopapular rash and desquamating erythema of the palms and soles; hepatosplenomegaly; and jaundice. [dartmouth.edu]

• Hepatomegaly

  We present an unusual case, in which a woman presenting with markedly decreased fetal movements at 29 weeks gestation following a recent increase in fundal height was noted sonographically to have fetal hydrops consisting of scalp edema, marked hepatomegaly [ncbi.nlm.nih.gov]

  Congenital SyphilisHydropsfetalis Nasal discharge Petechial rash Necrotizing funisitis within the matrix of the umbilical cord Hepatomegaly Rash Ostitis, Metaphysitis, P eriostitis Wimberger sign 12. [slideshare.net]

  If untreated, the infection may cause deafness, blindness, crippling, or death. congenital syphilis Congenital lues, fetal syphilis Neonatology Transplacental infection with Treponema pallidum Clinical Early–hepatomegaly, irritability, FTT, fever, perioral [medical-dictionary.thefreedictionary.com]

• Saber Shin

  shins, neurologic impairment, deafness, blindness Lab ↑ liver enzymes, anemia, monocytosis Diagnosis Serologic tests performed at birth may be negative. [medical-dictionary.thefreedictionary.com]

  shin Bilateral forward bowing of tibia is conclusive evidence for acquired, tertiary syphilis although similar changes can occur with congenital form Acquired syphilis affects skull (mixed sclerosis and lysis) Clavicle and tibia [learningradiology.com]

  Inflammation of bone's periosteum can lead to a number of morphological changes such as sinking in of the nose, resulting in a "Saddle Nose", or bowing of the tibia, resulting in "Saber Shins". [pathwaymedicine.org]

  Late manifestations (age 3 or older) result from scarring during early systemic disease and include Hutchinson teeth, Mulberry molars, abnormal facies, cranial nerve VIII deafness, bony changes such as saber shins and perforations of the hard palate, [aao.org]

• Arthralgia

  Although rare, bow legs, recurrent fever, anemia, and inflammatory arthralgias may be manifestations of late CS. Congenital syphilis should be considered throughout early childhood, especially if history of syphilis infection is present. [ncbi.nlm.nih.gov]

  DGI often results in petechial or pustular acral skin lesions, asymmetrical arthralgia, tenosynovitis, or septic arthritis. The infection is complicated occasionally by perihepatitis and rarely by endocarditis or meningitis. [cdc.gov]

• Corneal Opacity

  Eyes revealed dense corneal opacities with complete loss of vision in right eye. Left eye was normal. Patient was edentulous. VDRL test was reactive in dilution and TPHA was positive. Ultra sound showed hepatosplenomegaly with left renal cyst. [ijdvl.com]

• Hydrops Fetalis

  Early, intrauterine infection may not even be symptomatic at birth, but can also lead to preterm birth, hydrops fetalis or still birth. [symptoma.com]

  Congenital Syphilis Syphilis in pregnancy increases the risk of adverse outcomes, including pre-term delivery, low birth weight, non-immune hydrops fetalis, intrauterine growth restriction, stillbirth, and congenital syphilis. 26 ,, While 20% of infants [medicalreview.columbia.edu]

  TABLE 3 Common features of congenital syphilis* Feature Usual timing Details Spontaneous abortion/ stillbirth/ hydrops fetalis Any gestation Occurs in approximately 40% of cases if syphilis acquired during pregnancy, with risk being highest for first-trimester [cps.ca]

• Skin Rash

  Here we report a case of a one-month infant who presented with skin rash. [ncbi.nlm.nih.gov]

  Congenital syphilis may feature severe early skin rashes, often occurring in the first 10 weeks of life, bone and cartilage defects, liver and kidney disturbances, damage to the corneas (interstitial keratitis), deafness, peg teeth, saddle-shaped nose [medical-dictionary.thefreedictionary.com]

  Clinical manifestations may include hepatosplenomegaly, skin rash, lymphadenopathy and anemia. Bony changes of congenital syphilis predominately involve the bone metaphyses. [pedsradiology.com]

• Skin Lesion

  Pathologic examination of the skin lesion showed scattered dyskeratotic cells in the epidermis and interface dermatitis consistent with erythema multiforme. No spirochete could be found in the skin sections staining with Warthin-Starry stain. [ncbi.nlm.nih.gov]

  Early signs are characteristic skin lesions, lymphadenopathy, hepatosplenomegaly, failure to thrive, blood-stained nasal discharge, perioral fissures, meningitis, choroiditis, hydrocephalus, seizures, intellectual disability, osteochondritis, and pseudoparalysis [msdmanuals.com]

  The severe type (Oroya): The only skin manifestations are mild skin lesion due to insect bite at the site of inoculation. This type is acute followed by a chronic course presenting with verrucous skin lesions. [drmhijazy.com]

• Blister

  The male infant (2899 g) had normal vital signs, conjunctival congestion, splenohepatomegaly, and maculopapular rash with small blisters over the entire body. Serological tests on the infant and mother confirmed CS. [ncbi.nlm.nih.gov]

  Symptoms Symptoms in newborns may include: Failure to gain weight or failure to thrive Fever Irritability No bridge to nose (saddle nose) Rash of the mouth, genitals, and anus Rash -- starting as small blisters on the palms and soles, and later changing [nicklauschildrens.org]

  Enlarged liver and/or spleen (mass in belly) Failure to gain weight or failure to thrive (including prior to birth, with low birthweight) Fever Irritability Irritation and cracking of skin around the mouth, genitals, and anus Rash starting as small blisters [mountsinai.org]

• Petechiae

  Du Bois sign, narrowing of the little finger poorly developed maxillae. enlarged liver. enlarged spleen. petechiae. other skin rash. sabre shins. anemia. lymph node enlargement. jaundice. pseudoparalysis. snuffles, aka "syphilitic rhinitis", which appears [en.wikipedia.org]

  These include: Fever Anemia Jaundice Deafness Irritability Petechiae Skin rashes Sabre shins Joint swelling Enlarged liver Frontal bossing Enlarged spleen Pseudoparalysis Abnormal x-rays Lymph node enlargement Poorly developed maxillae Failure to gain [hxbenefit.com]

  They include skin lesions such as blisters and copper-colored rash on palms and soles, papules in face and lower abdomen and petechiae. Children often develop a purulent rhinitis and parents frequently report their children to snuffle. [symptoma.com]

  Petechiae may be present with severe thrombocytopenia. Most instances of neurosyphilis in neonates are asymptomatic, with diagnosis based on abnormal cerebrospinal fluid (CSF) results (see below). [clinicaladvisor.com]

• Eczema

  People with eczema also may be particularly susceptible to bacterial, viral, and fungal skin infections. [niaid.nih.gov]

  […] less commonly, tuberculosis, chancroid Anal ulcers : genital herpes, anal fissure, bacterial infections, trauma, inflammatory bowel disease Mouth ulcers : herpes simplex (cold sore), aphthous ulcers, trauma Rash Drug eruption Pityriasis rosea Psoriasis Eczema [dermnetnz.org]

• Frontal Bossing

  Mulberry molars (permanent first molars with multiple poorly developed cusps). frontal bossing. [en.wikipedia.org]

  History / PE : Early manifestations Hepatosplenomegaly Cutaneous lesions on palms/soles Jaundice/anemia Rhinorrhea Late/untreated manifestations Frontal bossing Hutchinson teeth Saddle nose Perioral fissures Diagnosis : VDRL FTA-ABS Treatment : Penicillin [medlibes.com]

  These include: Fever Anemia Jaundice Deafness Irritability Petechiae Skin rashes Sabre shins Joint swelling Enlarged liver Frontal bossing Enlarged spleen Pseudoparalysis Abnormal x-rays Lymph node enlargement Poorly developed maxillae Failure to gain [hxbenefit.com]

  Bony changes include sclerosing lesions, saber shin, frontal bossing and gummatous or destructive lesions within long bones. [hon.ch]

  Late, including stigmata: interstitial keratitis, Clutton's joints (arthritis of the knees), Hutchinson's incisors, mulberry molars, high palatal arch, rhagades, deafness, frontal bossing, short maxilla, protuberance of mandible, saddle nose deformity [patient.info]

• Seizure

  Early signs are characteristic skin lesions, lymphadenopathy, hepatosplenomegaly, failure to thrive, blood-stained nasal discharge, perioral fissures, meningitis, choroiditis, hydrocephalus, seizures, intellectual disability, osteochondritis, and pseudoparalysis [msdmanuals.com]

  But if the disease goes untreated, the babies can develop cataracts, deafness or seizures, and could die, according to the CDC. [scpr.org]

  Seizures are when the whole body or parts of the body move without control. Developmental delays. Developmental delays are when your child doesn’t reach developmental milestones when expected. [marchofdimes.org]

  Babies exposed in utero can have deformities, delays in development, or seizures along with many other problems such as rash, fever, an enlarged liver and spleen, anemia, and jaundice. [en.wikipedia.org]

Maternal serum is routinely screened in early and late pregnancy and at delivery and positive results are generally the first indication of CS. Nontreponemal and consecutive treponemal serum tests of the neonate may further confirm suspected CS. Those serum tests should not be carried out with cord blood due to little sensitive and specific results. Positive results should definitely prompt a thorough examination of the neonate for skin and mucosal lesions. Possibly detected lesions, the umbilical cord and the placenta should be further examined by darkfield microscopy and immunohistochemically. Characteristic pathological alterations may be found in all those tissues. CS can be confirmed if spirochetes are detected in those samples [12] [13]. Negative results do not rule out CS.

Infants and children developing symptoms of CS should also be thoroughly examined. A blood screen including liver function tests should be carried out as well as CSF analysis. Further neurologic testing may be indicated and comprise neuroimaging, brain stem and ophthalmologic tests. The long bones should be checked for signs of inflammatory alterations by means of radiographic imaging.

In some cases, CS manifests more than two years after birth. In order to diagnose CS, the child's and mother's medical history have to be taken into account as well as the results of a clinical examination and serologic tests. Hutchinson incisors, interstitial keratitis and eighth cranial nerve deafness are diagnostic for CS.

Even if CS cannot be confirmed, children at high risk of infection or those developing symptoms should be treated accordingly. This especially applies to neonates who often do not show any symptoms or only unspecific signs of illness [14].

The treatment of choice is penicillin. After successful treatment, nontreponemal tests should yield negative results. Treponemal tests, however, are based on the presence of antibodies and remain positive even after elimination of the pathogen [15].

The therapy of choice equals the treatment administered in confirmed cases and consists in i.v. administration of 50,000 units aqueous crystalline penicillin G per kg of body weight, every 12 hours during the first week of life and every 8 hours thereafter for a total of 10 days. An alternative therapy is i.m. administration of 50,000 units procaine penicillin G per kg of body weight, once a day and for 10 days. In order to eliminate all causative pathogens, continuous treatment is necessary. Thus, the whole treatment has to be repeated if more than one day is missed.

Treatment is strongly recommended for neonates at high risk of infection although they may not show CS symptoms at birth. Neonates are considered at high risk if their mother suffers from untreated syphilis, if treatment has not been appropriate or was carried out with unknown drugs. If there's a sudden increase in the maternal antibody titer, a relapse has to be suspected and the child is also considered at high risk. In case a full treatment is not an option for neonates at high risk, i.m. administration of a single dose of 50,000 units of benzathine penicillin per kg body weight may be an alternative only if the mother is clinically well, does not show any signs of infection and the child will be monitored as closely as possible. This regimen may also be applied as a precaution to neonates who are not at high risk because their mother has been adequately treated.

The treatment is slightly different for children who are diagnosed after four weeks of age or who develop late CS. While drug, single dose and time frame are equal to those applied in early CS (50,000 units of aqueous crystalline penicillin G per kg body weight over 10 days), i.v. applications have to be carried out every 4 to 6 hours. This increase in frequency of administration and therefore of overall dose is due to the risk of neurosyphilis in this group of patients [16]. A single dose of benzathine penicillin G may be administered as described above after completion of therapy. Similarly to neonates being born by adequately treated mothers, asymptomatic infants or children may be preventively treated with i.m. applications of benzathine penicillin G (50,000 units per kg of body weight, i.m., once a week for three weeks). For treatment of interstitial keratitis, an ophthalmologist should be consulted. It usually consists in corticosteroids and atropine.

Prognosis strongly depends on time passed between maternal infection and pregnancy. Because syphilis is considered to be much more contagious in the primary stage, a condition that is already attenuated but still present in the secondary stage, it is particularly unfavorable if the infection of the mother takes place a short time before or during pregnancy. On the other hand, longer times between maternal infection and pregnancy generally improve the outcome.

Untreated primary or secondary stage syphilis during pregnancy may lead to neonatal death (14%), stillbirth (25%) or CS (41%). The remaining 20% of infants are not infected and may be born healthy. If the infected mother did already pass these early stages of syphilis, which generally means that two years have passed since infection, these numbers reduce to 9%, 12% and 2% for neonatal death, stillbirth and CS, respectively. Three out of four children are born healthy [8]. Similar numbers have been reported elsewhere [9].

CS is an infectious disease caused by Treponema pallidum, a spirochete bacterium transmitted transplacentally during pregnancy or possibly by contact to maternal lesions at birth [4]. It has been estimated that up to 80% of children exposed to Treponema pallidum do indeed develop CS.

Consequences for the unborn or infant presumably depend on the time of infection of the mother. Early, intrauterine infection may not even be symptomatic at birth, but can also lead to preterm birth, hydrops fetalis or still birth.

According to estimations published by the World Health Organization (WHO), annually about 2,000,000 pregnant women get infected with Treponema pallidum [5]. Considering the aforementioned number of up to 80% of exposed fetuses becoming infected, one would estimate 1,600,000 children to be born with CS.

CS is a major public health problem in developing countries, particular in sub-Saharan Africa and subtropical Asia. Some studies report augmented case numbers in the Western world. Prenatal screenings and treatment of affected mothers would be desirable, may, however, not be available in all developing countries.

The spirochete bacterium Treponema pallidum is the causative agent of CS. Particularly women with primary and to a lesser degree those with secondary syphilis are at high risk to transmit the disease to their unborn children. Transmission is possible at all times during gestation. Children generally show symptoms of CS during the first two years of their life, only in rare cases do symptoms manifest later, a condition which is termed late CS [6]. Prenatal infection with Treponema pallidum can provoke neonatal death and stillbirth or may lead to severe disabilities such as neurologic problems, deafness and bone deformities [7]. Even though embryonal organogenesis is usually unaffected by infections with Treponema pallidum, CS is accompanied with systemic complications similar to those observed in secondary stage adult syphilis. All organ systems may thus be affected by CS.

Contrary to classical CS that develops in children aged under two years, late CS is not considered contagious.

Routine testing of pregnant mothers and, in case of positive results, consecutive treatment against Treponema pallidum often cures mother and child. The WHO does recommend such screenings to be part of the routine examinations done in the first and last trimester of pregnancy [17]. Only in a few cases that are not diagnosed and treated until late pregnancy children may still show symptoms of CS at birth.

At birth, another routine screen should be carried out. Children at risk of infection should be monitored closely. In order to identify those children at risk, improved surveillance and follow-up data would be desirable [18].

Serologic monitoring is particularly important for mothers with a medical history of syphilis. Increases in antibody titers may indicate a possible relapse and thus prompt treatment. Reinfections are also possible.

Since syphilis is a contagious infectious disease, family members of the patient should be tested clinically and serologically for signs of infection. It may be the case that the patient diagnosed with syphilis is not the pregnant woman herself but someone she had sexual intercourse with. The probability that the woman did acquire syphilis is about 25 to 50%. Thus, she should be treated.

Of note, medical personnel or other people in close contact with patients suffering from early CS may get infected. They should be examined for possible lesions two to three weeks afterwards and serologically screened at this point in time as well as three months later. They should be treated if necessary. After close, unprotected contact with the affected child before he or she was diagnosed with CS, even immediate treatment may be indicated.

Congenital syphilis (CS) results from intrauterine infection with Treponema pallidum, causative agent of syphilis. Mothers suffering from stage one or two syphilis do very likely transmit the infection to their unborn child. This may result in neonatal death, stillbirth. If a living child is born, he or she may show some signs of CS, but infants usually develop first symptoms some weeks after birth. Late CS is a rare variant of CS and affects children older than two years [1]. Symptoms comprise skin and mucosal lesions, hematological alterations and hepatomegaly, bone lesions and CNS symptoms.

Routine screens during pregnancy allow identification of infected mothers. Penicillin treatment is effective and generally cures mother and child. Despite those preventive measures, CS continues to concern health care providers and case numbers are increasing in developing and even in some developed countries [2] [3].

Children are at risk of developing congenital syphilis (CS) if their mother is infected with Treponema pallidum, causative agent of syphilis. She may transmit the disease to her unborn child if she is not or only inadequately treated. Increasing case numbers of CS have been observed in some industrialized countries, presumably due to lessening awareness among future parents. 

The disease may lead to stillbirth, life-threatening illness or severe disability in affected children. In order to prevent these consequences, routine screens should be performed during early and late pregnancy and at delivery. An effective treatment is available and consists in the application of antibiotics.

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