Congenital syphilis

Congenital syphilis develops as a result of the penetration of Trepopema pallidum into the fetus from a sick mother through a placenta affected by syphilis.

A healthy placenta is a filter for pale treponemas. For the spirochete to penetrate the fetus, the placenta must first be affected by syphilis, followed by a breach of the placental barrier. Fetal infection through the placenta can occur either when pale treponemas are carried into the child's body as emboli through the umbilical vein, or when pale treponemas penetrate the fetus's lymphatic system through the lymphatic slits of the umbilical cord.

The impact of syphilis on pregnancy is expressed in its disruption in the form of late miscarriages and premature births, with stillbirths (premature or on time) and the birth of sick children often occurring.

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Symptoms of congenital syphilis

Taking into account clinical manifestations from an epidemiological point of view, the following periods of congenital syphilis are distinguished: fetal syphilis, early congenital syphilis (in which syphilis of infancy and syphilis of early childhood are distinguished) and late congenital syphilis (after 4 years).

In fetal syphilis, specific damage to internal organs and systems is observed, which leads to late miscarriages and stillbirths.

The dead fetus has a characteristic appearance: the skin is flabby, macerated due to underdevelopment of the subcutaneous tissue, easily gathers into small folds, the face is wrinkled and acquires an old-age appearance (the face of an old man). Enlargement of the liver, spleen and signs of white pneumonia are noted.

Clinical manifestations of congenital syphilis in infancy occur during the first 2 months of life. The skin, mucous membranes and internal organs are affected simultaneously.

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

At 8-10 weeks after birth, diffuse Hochsinger's infiltration appears, which is usually localized on the soles, palms, face and scalp. Then the characteristic features of the disease develop: the lesion is sharply delimited, has a smooth, shiny, bluish-red, then cracked brownish-red surface, is characterized by a dense-elastic consistency, which leads to the formation of cracks, which have radial directions around the mouth and leave lifelong so-called radiant Robinson-Fournier scars. In addition, widespread or limited roseolous, papular and pustular rashes in all their varieties are observed, similar to those in the secondary period of syphilis. These roseola have a tendency to merge and peel. The general condition of the child is impaired (fever), there is small focal or diffuse hair loss, and the development of syphilitic rhinitis (narrowing of the nasal passages, mucopurulent discharge drying into crusts). Breathing through the nose is greatly hampered, making sucking impossible. Papular infiltration of the nasal septum leads to its destruction and deformation of the nose (in the form of a saddle or blunt, "goat-like"). There is damage to the skeletal system in the form of osteochondritis, ending in pathological fractures of the bones of the extremities (Parrot's pseudoparalysis).

In congenital syphilis of early childhood, limited large-papular (usually weeping) rashes of the broad condylomas type are most often observed on the skin, and erosive papules on the mucous membranes; bones are often affected (syphilitic periostitis of long tubular bones), and less often, internal organs and the nervous system.

Manifestations of late congenital syphilis occur between the ages of 5 and 17, but may also appear later. Symptoms of late congenital syphilis can be divided into "definite", "probable" and "dystrophic" signs and often correspond to the damage to various organs and systems in acquired tertiary syphilis.

The unconditional signs include Hutchinson's triad: Hutchinson's teeth (barrel-shaped or chisel-shaped incisors, hypoplasia of the chewing surface with a crescent-shaped notch along the free edge); parenchymatous 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 degenerative processes in the auditory nerve).

Possible signs include: syphilitic chorioretinitis (characteristic "salt and pepper" pattern on the fundus); saber-shaped shins - result of diffuse osteoperiostitis with reactive osteosclerosis and anterior curvature of the leg bones; saddle-shaped or "goat-like" nose (result of syphilitic rhinitis or gumma of the nasal septum); breech skull (sharply protruding frontal tubercles with a groove located between them); "kidney-shaped (purse-string) tooth", Myna's tooth (underdevelopment of the chewing tubercles of the first molars); Fournier's "pike tooth" (similar change in the canine with thinning of its free end); Robinson-Fournier radial scars (around the mouth after Hochsinger infiltrations); syphilitic gonitis (Cleston symovitis), occurring along; type of chronic allergic synovitis (characterized by the absence of sharp pain, fever and joint dysfunction); damage to the nervous system (speech disorders, dementia, etc.).

Dystrophic signs include: Ausitid's sign (thickening of the sternal end of the clavicle due to diffuse hyperostosis); "Olympic forehead" (enlargement of the frontal and parietal tubercles); high ("Gothic") palate; infantile (shortened) little finger of Dubois-Gissart (hypoplasia of the fifth metacarpal bone); Queyrat's axiphoidia (absence of the xiphoid process); Gachet's diastema (widely spaced upper incisors); Carabelli's tubercle (additional tubercle on the chewing surface of the first molar of the upper jaw); Tarpovsky's hypertrichosis (overgrowth of hair on the forehead almost to the eyebrows). All of the listed dystrophies do not have diagnostic value individually. Only the presence of several dystrophies in combination with other signs of syphilis and anamnesis data can help to diagnose congenital syphilis in unclear cases.

Diagnosis of congenital syphilis

Diagnosis of congenital syphilis is complicated by the possibility of transplacental transfer of maternal IgG to the fetus. This complicates the interpretation of a positive serologic test for syphilis in the infant. The decision to treat must often be based on the identification of syphilis in the mother, the adequacy of treatment of the mother, the presence of clinical, laboratory, or radiographic evidence of syphilis in the infant, and a comparison of the infant's nontreponemal serologic test result with that of the mother.

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Who needs to be examined?

All infants born to seropositive mothers should have quantitative nontreponemal serologic tests (RPR or VDRL) performed on serum (umbilical cord blood may be contaminated with maternal blood and give a false-positive result). Treponemal tests TRHA and FTA-abs should not be performed on infant serum.

Survey

All infants born to mothers with positive serologic tests for syphilis should have a thorough physical examination to detect signs of congenital syphilis (e.g., protein-free edema, jaundice, gelatosplenomegaly, rhinitis, skin rash, and/or pseudoparalysis of the extremities). Immunofluorescence is suggested to detect placental or umbilical cord pathology. Darkfield microscopy or DIF is also recommended for examination of suspicious lesions or discharge (e.g., nasal discharge).

Further evaluation of the infant depends on the findings of any abnormalities on physical examination, the maternal treatment history, the stage of infection at the time of treatment, and a comparison of nontreponemal test titers of the mother (at the time of delivery) and the infant, performed using the same methods and in the same laboratory.

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Treatment of congenital syphilis

All infants should be treated prophylactically for congenital syphilis if they are born to mothers who:

• had untreated syphilis at the time of delivery (women treated with a regimen other than that recommended in this guideline should be considered untreated); or
• after treatment, relapse or reinfection was observed, confirmed by serological tests (an increase in non-treponemal test titers by more than 4 times); or
• treatment of syphilis during pregnancy was carried out with erythromycin or other non-penicillin drugs (the absence of a 4-fold increase in titers in the child does not exclude the presence of congenital syphilis), or
• treatment for syphilis was carried out less than 1 month before delivery, or
• the medical history does not reflect the fact of treatment for syphilis, or
• despite treatment of early syphilis during pregnancy with penicillin according to the appropriate regimen, non-treponemal test titers did not decrease by more than 4 times, or
• appropriate treatment was administered before pregnancy, but there was insufficient serologic monitoring to ensure an adequate response to treatment and the absence of current infection (a satisfactory response includes a) a more than 4-fold decrease in nontreponemal test titers in patients treated for early syphilis, b) stabilization or a drop in nontreponemal titers to a level less than or equal to 1:4 for other patients).

Evaluation of infants who, despite treatment of the mother for syphilis, have abnormal physical examination findings, namely, features consistent with congenital syphilis or a 4-fold increase in qualitative nontreponemal test titers compared with those of the mother (absence of a 4-fold increase in titers in the infant does not indicate the absence of congenital syphilis) or a positive dark-field microscopy or positive DFT with body fluids, should include:

• CSF examination: VDRL, cytosis, protein;
• clinical blood test and platelet count;
• other studies when clinically indicated: (eg, long bone radiography, chest radiography, liver function tests, skull ultrasound, ophthalmologic examination, examination of the auditory center of the brainstem).

Recommended treatment regimens for syphilis

Water-soluble crystalline penicillin G,

100,000-150,000 IU/kg/day (administer 50,000 IU/kg intravenously every 12 hours

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

Or Procaine penicillin G, 50,000 U/kg intramuscularly once daily for 10-14 days.

If treatment is interrupted for more than 1 day, the full course is repeated. Clinical experience with the use of other antibacterial drugs such as ampicillin is insufficient. When possible, a 10-day course of penicillin is preferable. When using drugs other than penicillin, careful serological monitoring is required to assess the adequacy of treatment.

In all other situations, a history of syphilis and its treatment in the mother is an indication for examination and treatment of the child. If infants with normal physical examination results have titers of qualitative nontreponemal serologic tests the same as or 4 times lower than the mother's, then the decision to treat the child depends on the stage of the mother's disease 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 entry in the medical history, or she has received treatment with non-treponemal drugs less than 4 weeks before delivery, b) the adequacy of treatment in the mother cannot be assessed because there has been no 4-fold drop in non-treponemal test titers, c) there is a suspicion of relapse/reinfection due to a four-fold increase in non-treponemal test titers in the mother.

Interpreting CSF results in neonates can be difficult: normal values vary with gestational age and are higher in premature infants. Healthy neonates may have values as high as 25 WBC/mm and 150 mg protein/dL; however, some experts recommend lower values (5 WBC/mm and 40 mg protein/dL) as the upper limit of normal. Other causes that may cause high values should also be considered.

Treatment regimens:

• water-soluble penicillin G or procaine penicillin as above for 10 days. Some experts prefer this treatment in cases where the mother has not been treated for early syphilis at the time of delivery. Testing for cure is not required if parenteral treatment has been given for the 10 days indicated. However, such evaluation may be helpful; lumbar puncture may reveal abnormal CSF, which may require careful monitoring. Other tests, such as a hemogram, platelet count, and bone radiography, may be done to further confirm the diagnosis of congenital syphilis;

Or

• benzathine penicillin G, 50,000 U/kg IM once - in children without abnormalities after a full examination (CSF examination, bone radiography, hemogram with platelet count), after which control is recommended. If pathology is detected during examination of the infant or it was not performed, or the CSF analysis cannot be interpreted as blood contamination, then in such cases a 10-day course of penicillin is required in accordance with the above treatment regimen.
• The infant should be given benzathine penicillin G, 50,000 U/kg IM once, if the mother was treated: a) during pregnancy, according to the stage of the disease and more than 4 weeks before delivery, b) for early syphilis and nontreponemal serologic test titers have decreased by a factor of 4, or c) for late latent syphilis and nontreponemal test titers have remained stable or decreased and there is no evidence of relapse or reinfection in the mother. (Note: Some practitioners do not treat such infants but perform careful serologic monitoring.) In such situations, if the infant's nontreponemal test results are negative, no treatment is needed.
• Treatment of infants is not performed if the mother was treated before pregnancy and, with repeated clinical and serologic monitoring, nontreponemal serologic test titers 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 equal to 1:4). Some experts prescribe benzathine penicillin G, 50,000 U/kg IM once in such cases, especially if there is no guarantee that subsequent monitoring will be performed.

Diagnosis and treatment of congenital syphilis in infants and older children

If children have positive serological tests for syphilis after the neonatal period (after 1 month of life), the mother's serological status and previous test results should be determined to assess whether the child has congenital or acquired syphilis (if acquired, see Primary and Secondary Syphilis and Latent Syphilis). If congenital syphilis is suspected, the child should undergo a full evaluation: CSF examination for cell count, protein and VDRL (CSF results are considered abnormal if VDRL is positive, cytosis is more than 5 leukocytes/mm and/or protein is > 40 mg/dL); eye examination, other tests such as long bone radiography, hemogram, platelet count, hearing examination* if clinically indicated. Any child suspected of having congenital syphilis or who has neurologic symptoms should be treated with aqueous crystalline penicillin G, 200,000-300,000 units/kg/day IV (50,000 units/kg every 4-6 hours) for 10 days.

**If the infant has negative nontreponemal titers and the likelihood of infection is low, some experts recommend giving benzathine penicillin G, 50,000 units/kg IM as a single dose in case the infant is incubating, followed by careful serologic monitoring.

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Follow-up observation

All infants with positive serologic tests for syphilis (or an infant whose mother tested positive for serologic tests for syphilis before delivery) should be closely monitored and have serologic testing (nontreponemal testing) every 2 to 3 months until test results become negative or decrease by a factor of 4. Nontreponemal titers should decrease by 3 months of age and become negative by 6 months unless the infant was infected (positive titers resulted from passive transfer of IgG antibodies from the mother) or was infected but received adequate treatment (response to treatment may be delayed if the infant was treated after the neonatal period). If titers are found to remain stable or to increase from 6 to 12 months, the infant should be retested with CSF testing and given a full 10-day course of parenteral penicillin G.

Treponemal tests are not recommended for assessing response to treatment because if the child has been infected, the results may remain positive despite successful therapy. Antibodies to treponemes passively transferred from the mother may be detectable up to 15 months of age. If positive treponemal tests are detected in a child over 18 months of age, syphilis is classified as congenital. If nontreponemal tests are negative by this age, no further testing or treatment is required. If nontreponemal tests are positive by 18 months, the child should be re-examined and treated for congenital syphilis.

Children with initial CSF abnormalities should have their CSF examined again every 6 months until the results return to normal. Finding a positive CSF VDRL or CSF abnormalities that cannot be caused by other diseases are indications to retreat the child for possible neurosyphilis.

Follow-up of children treated for congenital syphilis after the neonatal period should be the same as for neonates.

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Special Notes

Allergy to penicillin in the treatment of syphilis

Infants and children requiring antisyphilitic treatment who are allergic to penicillin or who develop an allergic reaction presumably to penicillin derivatives should be treated with penicillin after desensitization if necessary. Skin testing may be useful in some patients under certain circumstances (see Management of patients with penicillin allergy). There are insufficient data on the use of other antimicrobial agents such as ceftriaxone; careful serologic monitoring and CSF examination are necessary when nonpenicillin agents are used.

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HIV infection and syphilis

There is no evidence that infants with congenital syphilis whose mothers are coinfected with HIV require any special testing, treatment, or monitoring for syphilis compared with all other infants.

Effective prevention and detection of congenital syphilis depends on detection of syphilis in pregnant women and, therefore, on routine serologic screening at the first visit during pregnancy. In groups and populations considered to be at high risk for congenital syphilis, serologic testing and sexual history should be obtained at 28 weeks of pregnancy and at delivery. In addition, information regarding the treatment of the sexual partner should be obtained to assess the likelihood of reinfection in the pregnant woman. All pregnant women with syphilis should be tested for HIV infection.

Serologic testing of maternal serum is recommended, but not routine screening of serum or cord blood in newborns, because the infant's serologic test may be negative if the mother has low titers or was infected late in pregnancy. No infant should be discharged from hospital without maternal serologic testing documented at least once during pregnancy.

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