Congenital syphilis

Congenital syphilis develops as a result of getting a pale trepidemia in the fetus from a sick mother through a placenta afflicted with syphilis.

A healthy placenta is a filter for pale treponemes. To penetrate the spirochaete into the fetus, a pre-infection of the placenta with syphilis is necessary, followed by a violation of the placental barrier. Infection of the fetus through the placenta can occur either when pale treponema is introduced into the child's body as emboli through the umbilical vein, or when pale treponema penetrates the lymphatic system of the fetus through the lymphatic fissures of the umbilical cord.

Influence of syphilis on pregnancy is expressed in the violation of its course in the form of late miscarriages and premature birth, and often there are stillbirths (premature or on time), the birth of sick children.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10]

Symptoms of congenital syphilis

Given the clinical manifestations from an epidemiological point of view, the following periods of congenital syphilis are distinguished: syphilis of the fetus, early congenital syphilis (it secrete syphilis of infancy and syphilis of early childhood) and late congenital syphilis (after 4 years).

With syphilis of the fetus, a specific lesion of internal organs and systems is noted, which leads to late miscarriages and stillbirths.

The fatal fruit has a characteristic appearance: the skin is flabby, macerated due to underdevelopment of the subcutaneous tissue, it is easily collected into small folds, the face is wrinkled and acquires an old age (old man's face). There is an increase in the liver, spleen and the phenomenon of white pneumonia.

Clinical manifestations of congenital syphilis of infancy occur during the first 2 months of life. At the same time, the skin, mucous membranes and internal organs are affected.

The earliest rash in this period is syphilitic pemphigus. Eruptions are located on the palms, soles, forearms and legs. On the infiltrated base, bubbles of the size of a pea and cherry appear, at first their contents are serous, then becomes purulent, sometimes hemorrhagic. Bubbles are surrounded by a zone of a specific papular infiltrate of cyanotic red color.

At 8-10 weeks after birth, diffuse infiltration of Gohsinger appears, which is usually localized on soles, palms, face and scalp. Then the characteristic features of the disease develop: the lesion is sharply delimited, initially smooth, shiny, cyanotic red, then cracked brownish-red surface, characterized by a dense elastic consistency, which leads to the formation of cracks that have radial directions in the mouth circumference and are left for life so called the Rayon-Fournier rays. In addition, widespread or limited roseose, papular and pustular eruptions are observed in all their varieties, similar to those in the secondary stage of syphilis. These roseols are prone to fusion and peeling. There is a violation of the general condition of the child (fever), small focal or diffuse hair loss, the development of syphilitic rhinitis (narrowing of the nasal passages, mucopurulent discharge, shrinking into the crust). Breathing through the nose is greatly hampered, which makes the act of sucking impossible. Papular infiltration of the nasal septum leads to the destruction and deformation of the nose (in the form of saddle or blunt, "goat"). There is a lesion of the osteochondritis bone system, which ends with pathological fractures of the limb bones (pseudo paralysis).

When congenital syphilis of early childhood on the skin are more often limited large-papilled (usually mopping) rashes such as wide condylomas, on the mucous membranes - erosive papules; often affected by bones (syphilitic periostitis of long tubular bones), less often - internal organs and nervous system.

Manifestations of late congenital syphilis occur between the ages of 5 and 17, but may appear later. Symptoms of late congenital syphilis can be divided into "unconditional", "probable" and "dystrophic" symptoms and often correspond to the defeat of various organs and systems with acquired tertiary syphilis.

Unconditional signs include the Getchinson triad: the Getschinson teeth (barrel-shaped or chisel-shaped incisors, hypoplasia of the masticatory surface with a semilunar recess along the free margin); parenchymal keratitis (uniform milky white opacity of the cornea with photophobia, lacrimation and blepharospasm); labyrinthine deafness (inflammatory phenomena and hemorrhages in the inner ear in combination with dystrophic processes in the auditory nerve).

Probable signs include: syphilitic chorioretinitis (a characteristic picture of "salt and pepper" on the fundus); saberiform shin - the result of diffuse osteoperiostitis with reactive osteosclerosis and curvature of the shins of the shins anteriorly; Saddle or "goat" nose (result of syphilitic cold or gum of nasal septum); buttock-like skull (sharply outstretched frontal hillocks with groove located between them); "Kidney-shaped (kisetoobrazny) tooth", Mya's tooth (underdevelopment of masticatory tubercles of the first molars); "Finge tooth" Fournier (a similar change of canine with thinning of its free end); the Robinson-Fournier scars (in the circumference of the mouth after the infiltration of Gochsinger); syphilitic gonits (Simvita Klstton), flowing through; type of chronic allergic synovitis (differ in the absence of sharp pain, fever and violations of joint function); lesions of the nervous system (speech disorders, dementia, etc.).

Dystrophic features include: a sign of Avsitidian (thickening of the sternal end of the clavicle due to diffuse hyperostosis); "Olympic forehead" (an increase in the frontal and parietal tubercles); high ("gothic") sky; infantile (shortened) little finger of Dubois-Gissar (hypoplasia of the fifth metacarpal bone); axiphoidia of Keira (absence of the xiphoid process); diastema Gachet (widely spaced upper incisors); the tubercle of Carabelli (an additional tubercle on the masticatory surface of the first molar of the upper jaw); hypertrichosis of Tarpovsky (overgrowth with forehead almost to the eyebrows). All these dystrophies do not have a diagnostic value alone. Only the presence of several dystrophies in combination with other signs of syphilis and anamnesis can help in unclear cases to diagnose congenital syphilis.

Diagnosis of congenital syphilis

Diagnosis of congenital syphilis is complicated by the possibility of transplacental transfer to the fetus of the mother IgG. This makes it difficult to interpret a positive serological test for syphilis in a baby. The decision to prescribe treatment should often be determined based on the detection of syphilis in the mother, the adequacy of maternal treatment, the presence of clinical, laboratory or radiographic signs of syphilis in the child and a comparison of the non-treponemal serological test result in the child with the result in the mother.

[11], [12], [13], [14], [15], [16], [17], [18]

Who needs to be examined?

All children born from seropositive mothers should be given quantitative non-treponemal serology tests (RPR or VDRL) with serum (the blood of the umbilical cord may be contaminated with maternal blood and give false positive results). Treponemal tests of TRNA (FGGA) and FTA-abs (RIF-abs) with the serum of the baby is not necessary.

Examination

All children born to mothers with positive serological response to syphilis should undergo a thorough physical examination to identify signs of congenital syphilis (eg, protein-free edema, jaundice, gelatomplenomegaly, rhinitis, skin rash and / or pseudo-paralysis of the limbs). To determine the pathology of the placenta or umbilical cord, it is suggested to use the method of immunofluorescence. Darkfield microscopy or UIF is also recommended for studies of suspicious lesions or secretions (eg, discharge from the nose).

Further examination of the baby depends on the results of the detection of any pathology in the physical examination, the history of the treatment of the mother, the stage of infection at a given time of treatment, and the comparison of the mother's non-treponemal tests (by the time of delivery) and the baby, conducted using the same methods and in one laboratory.

[19], [20], [21], [22], [23], [24], [25], [26]

Treatment of congenital syphilis

All babies should be prophylactically treated for congenital syphilis if they are born from mothers who have:

• at the time of delivery, there was untreated syphilis (women treated according to a different scheme, and not recommended in this manual should be considered untreated); or
• after treatment, serological tests confirmed relapse or reinfection (an increase in the titres of non-treponemal tests more than 4-fold); or
• treatment of syphilis during pregnancy was carried out with erythromycin or other drugs of the non-penicillin series (the absence of a 4-fold increase in titers in a child does not exclude the presence of congenital syphilis), or 
• Treatment of syphilis was performed less than 1 month before childbirth, or 
• the history of the disease does not reflect the fact of treatment of syphilis, or 
• despite treatment of early syphilis during pregnancy with penicillin according to the corresponding scheme, the titres of non-treponemal tests did not decrease more than 4-fold, or
• treatment was carried out before pregnancy, but there was insufficient serological control to ensure an adequate response to treatment and no infection at the present time (a satisfactory answer includes a) more than a 4-fold decrease in non-treponemal titres in patients treated with early syphilis, b ) stabilization or reduction of non-treponemal titers to a level less than or equal to 1: 4 for other patients). 

Examination of infants who, in spite of treatment for syphilis in their mother, showed abnormalities in a physical examination, namely, signs characteristic of congenital syphilis, or 4 times higher titres of qualitative non-treponemal tests, compared with those of the mother (absence of 4- fold increase in titers in an infant does not indicate the absence of congenital syphilis), or a positive microscopic result in a dark field or a positive MU with body fluids, should include:

• CSF study: VDRL, cytosis, protein; 
• the clinical analysis of blood and the calculation of the number of platelets; 
• other studies with clinical indications: (for example, radiography of long tubular bones, chest radiography, liver tests, ultrasound of the skull, ophthalmological examination, examination of the auditory center of the brainstem).

Recommended regimens for the treatment of syphilis

The water-soluble crystalline penicillin G,

100,000-150000 units / kg / day (enter 50,000 units / kg iv every 12 hours in

During the first 7 days of life and then every 8 hours) for 10-14 days

Or Procaine penicillin G, 50000 units / kg IM once daily for 10-14 days.

If the treatment was interrupted for more than 1 day, the full course is again conducted. Clinical experience on the use of other antibacterial drugs, such as ampicillin is not enough. If possible, a 10-day course of treatment with penicillin should be used. When using other drugs other than penicillin, careful serological testing is required to assess the adequacy of treatment.

In all other situations, the presence of syphilis and its treatment in the mother's anamnesis is an indication for the examination and treatment of the child. If babies with normal results of physical examination have titres of qualitative non-treponemal serological tests that are the same as those of a mother or 4 times lower, the decision to treat it depends on the stage of the disease in the mother and the course of her treatment.

The infant should be treated in the following cases: a) if the mother has not been treated, or there is no corresponding record in the case history, or she received treatment with non-lichenicin drugs less than 4 weeks before the birth, b) the adequacy of treatment in the mother .to. There was no drop in the non-treponemal test titre 4 times, c) there are suspicions of relapse / reinfection due to a fourfold increase in the titres of non-treponemal tests in the mother.

Interpretation of the results of the study of CSF in newborns can be difficult: the values of the rate vary depending on the timing of gestation and higher in premature infants. In healthy newborns, such high numbers as 25 leukocytes / mm and 150 mg of protein / dL can be observed; however, some experts recommend lower limits (5 white blood cells / mm and 40 mg protein / dL) as the upper limit of the norm. Other factors that may lead to high rates should also be considered.

Treatment regimens:

• water-soluble penicillin G or procaine-penicillin according to the above scheme for 10 days. Some specialists prefer to carry out this treatment in cases when the mother was not treated from early syphilis by the time of delivery. Control of cure is not required if parenteral treatment was performed during the indicated 10 days. However, such an assessment may be useful; with spinal puncture, it is possible to detect a pathology in the CSF, which may require careful monitoring. Other tests such as hemogram, platelet count and bone radiography can be performed to further confirm the diagnosis of congenital syphilis;

Or

• benzathine penicillin G, 50000 units / kg IM once - in children without deviations from the norm with a full examination (CSF study, radiography of the bones, hemogram counting the number of platelets), after which monitoring is recommended. If an abnormality is detected in the infant or not, or CSF analysis can not be interpreted as blood contamination, then a 10-day course of penicillin is required in accordance with the above treatment regimen.
• The infant should be prescribed benzathine penicillin G, 50000 units / kg IM once, if the mother was treated: a) during pregnancy, according to the stage of the disease and more than 4 weeks before the birth, b) about early syphilis and titres of non-treponemal serology tests decreased 4-fold or c) for late latent syphilis and the titres of non-treponemal tests remained stable or decreased and there are no signs characteristic of relapse or reinfection in the mother. (Note: some specialists do not treat such babies, but conduct thorough serological monitoring). In such situations, if the baby has non-treponemal test results that are negative, no treatment is needed. 
• Infants are not treated if the mother was treated prior to pregnancy and with multiple clinical and serological controls, the titres of non-treponemal serology tests remained low or stable before and during pregnancy, and at the time of delivery (VDRL less than or equal to 1: 2; RPR less than or is 1: 4). Some specialists prescribe in such cases benzathine penicillin G, 50000 units / kg IM once, especially if there is no guarantee that subsequent monitoring will be carried out. 

Diagnosis and treatment of congenital syphilis in infants and older children

If children have positive results of serological reactions to syphilis after the newborn period (after the first month of life), then the serological status of the mother and the results of previous studies should be clarified in order to assess whether the child has congenital or acquired syphilis (if syphilis is acquired , see sections Primary and secondary syphilis and latent syphilis). If a child is suspected of having congenital syphilis, he should be fully examined: the CSF study to count cells, protein, and VDRL (CSF results are considered pathological, with VDRL positive, cytosis greater than 5 leukocytes / mm and / or protein> 40 mg / dL); eye examination, other tests, such as radiography of long tubular bones, hemogram, platelet count, examination of * hearing organs if there are clinical indications. Any child suspected of having congenital syphilis or having neurological symptoms should be treated with aqueous crystalline penicillin G, 200,000-300,000 units / kg / day IV (50,000 U / kg every 4-6 hours) for 10 days.

** If the baby has non-treponemal test titers negative, and the likelihood of infection is small. Some experts recommend the administration of benzathine penicillin G, 50000 units / kg IM once in case the child has an incubation period, followed by careful serological control.

[27], [28], [29], [30], [31]

Follow-up

All children with positive serological reactions to syphilis (or a child whose mother had positive serologic responses to syphilis were determined before delivery) should be carefully monitored and serologically tested (non-treponemal tests) every 2-3 months until the test results will become negative or not decrease by 4 times. The titres of non-treponemal tests should decrease by 3 months and become negative by 6 months if the child has not been infected (positive titres were the result of passive transfer of IgG antibodies from the mother) or were infected but received adequate treatment (response to treatment may be slow, if the child received treatment after the neonatal period). If it is found that the titers remain stable or increase from the 6th to the 12th month, the child should be examined again with a CSF study and undergo a complete 10-day course of treatment with parenteral penicillin G.

It is not recommended to use treponemal tests to assess the response to treatment, because if the child has been infected, the results may remain positive, despite successful therapy. Passively transferred from the mother antibodies to treponemam can be determined before the age of 15 months. If positive reactions of treponemal tests are determined in a child older than 18 months, syphilis is classified as congenital. If non-treponemal tests are negative by this age, then further examination and treatment is not required. If by 18 months non-treponemal tests are positive, the child should be examined again and treated for congenital syphilis.

Children with initial abnormalities in CSF should undergo a CSF re-examination every 6 months before the results are normalized. Detection of positive VDRL in CSF or CSF deviation, if they can not be caused by other diseases, are indications for a re-treatment of a child from a possible neurosyphilis.

Further monitoring of children treated for congenital syphilis after the neonatal period should be the same as in newborns.

[32], [33], [34], [35], [36]

Special Remarks

Allergy to penicillin in the treatment of syphilis

Infants and children who need antisyphilitic treatment who are allergic to penicillin or develop an allergic reaction, presumably to penicillin derivatives, should be treated with penicillin after desensitization, if necessary. In some circumstances, it may be useful for some patients to have skin tests (see Management of patients with penicillin allergy). There is insufficient evidence of the use of other antimicrobial agents, such as ceftriaxone; with the use of drugs of the non-penicillin series, it is necessary to carry out a thorough serological control and a study of CSF.

[37], [38], [39], [40], [41], [42], [43], [44], [45]

HIV infection and syphilis

There is no evidence that newborns with congenital syphilis whose mothers are coinfected with HIV require any special examination, treatment or supervision for syphilis compared to all other children.

Effective prevention and detection of congenital syphilis depends on the detection of syphilis in pregnant women, and hence from routine serological screening at the first appearance during pregnancy. In groups and populations that are at high risk for congenital syphilis, a serological examination should be conducted and a sexual history recorded at week 28 of pregnancy and by the time of delivery. In addition, in order to assess the likelihood of reinfection of a pregnant woman, information should be obtained regarding the treatment of her sexual partner. All pregnant women with syphilis should be examined for HIV infection.

Serologic testing of maternal serum is recommended, but not routine screening of serum or blood from the umbilical cord of a newborn, since a serological test in an infant may be negative if its mother has low titers or was infected in late pregnancy. No child should be discharged from the hospital unless there is a serological test of his mother documented at least once during pregnancy.

Examination and treatment of the child in the first month of life.