congenital syphilis.pptx

congenital syphilis

1. Dr Nibin CONGENITAL SYPHILIS

2. Syphilis is a chronic systemic, sexually or vertically transmitted Easily treatable, if detected early Significant morbidity, if left unchecked

3. MICROBE.. Treponema pallidum, a spirochete Sub species : pallidum endemicum carateum Needs dark field ,phase contrast microscopy or direct immunoflurescent or silver staining or PCR

4. 2 forms of syphilis can occur in children and adolescents * Congenital * Acquired - Almost exclusively by sexual contact - Less commonly via contaminated blood or direct contact

5. CONGENITAL SYPHILIS Transplacental transmission or occasionally by intrapartum contact with infectious lesions ( active genital lesions ) Transmission can occur at any stage of pregnancy Can result in, Early fetal loss Preterm births, LBW Still births or neonatal deaths ~40% Congenital syphilis

6. Maternal factors influencing acquisition of infection Women with primary & secondary syphilis and spirochetemia are more likely to transmit the disease than those with latent infection Highest risk during 1 st 4 yrs of infection Limited access to health care Late or no prenatal care, inadequate Rx during pregnancy Inadequate serological response of mother to Rx Drug use Multiple sex partners, sex trade Unprotected sexual contact, incarceration Untreated syphilis during pregnancy  ~100 % vertical transmission : Obliterating endarteritis and profound effects on pregnancy outcome

7. CLINICAL FEATURES Majority are asymptomatic @ birth ( including upto 40 % with CSF seeding) : Identified only by routine prenatal screening Without Rx , symptomatic by weeks to months Among infants symptomatic at birth or in the first few months of life, manifestations have traditionally been divided into, Early stage Late stage

8. All stages of congenital syphilis are characterized by a vasculitis , with progression to necrosis and fibrosis. The early signs  A ppear during the first 2 yr of life (resulting from transplacental spirochetemia ) Late signs  gradually during the first 2 decades. Early manifestations vary and involve multiple organ systems - Analogous to the secondary stage of acquired syphilis. - Hepato-splenomegaly, jaundice, and elevated liver enzymes - Histologically, liver involvement includes bile stasis, fibrosis, - Extramedullary hematopoiesis. Lymphadenopathy tends to be diffuse and resolve spontaneously, although shotty nodes can persist.

9. Coombs-negative hemolytic anemia is characteristic. Thrombocytopenia is often associated with platelet trapping in an enlarged spleen. Osteochondritis and periostitis : Classic Erythematous maculopapular or vesiculobullous rashes f/b desquamation involving hands and feet Mucous patches, persistent rhinitis (snuffles ), and condylomatous lesions are highly characteristic features of mucous membrane involvement containing abundant spirochetes.

10. Blood and moist open lesions from infants with congenital syphilis are infectious until 24 hr of appropriate treatment

11. Bone involvement is common. Roentgenographic abnormalities include, Wimberger lines Painful osteochondritis at the wrists, elbows, ankles, and knees with irritability and refusal to move the involved extremity ( Pseudoparalysis of Parrot ) Periostitis of the long bones and rarely the skull.

12. Congenital neurosyphilis :often asymptomatic in newborns FTT Renal involvement include hypertension, hematuria,proteinuria,hypercholesterolemia , and hypocomplementemia (glomerular deposition of circulating immune complexes.) Less-common- gastroenteritis, peritonitis, pancreatitis, glaucoma,chorioretinitis Nonimmune hydrops testicular masses Other manifestations

13. Late manifestations (children > 2 yr of age) - rarely seen in developed countries. - due to chronic granulomatous inflammation of bone, teeth and CNS Skeletal changes – Due to persistent or recurrent periostitis . Dental abnormalities – Hutchinson teeth Repeated caries. Saddle nose

14. Late manifestations of congenital syphilis Hypersensitivity phenomena. - Clutton joint - U/L or B/L interstitial keratitis,choroiditis , retinitis - vascular occlusion, optic atrophy - Soft-tissue gummas , - paroxysmal cold hemoglobinuria

15. DIAGNOSIS Results must always be interpreted in the context of patient history and physical examination. Darkfield microscopy to demonstrate the microbe or direct fluorescent antibody testing : specimens from skin lesions, placenta or umbilical cord PCR Despite the absence of a true gold standard serologic assay, serologic testing for syphilis remains the principal

16. 2-step screening process nontreponemal test followed by a confirmatory treponemal test The Venereal Disease Research Laboratory (VDRL) and rapid plasma reagin (RPR)  N ontreponemal tests : screening and in monitoring therapy. Nontreponemal tests - Usually nonreactive within 1 yr of adequate therapy for primary syphilis and within 2 yr of for secondary disease. 15–20% of patients become serofast ( nontreponemal titers persisting at low levels for long periods).

17. In congenital infection, these tests become nonreactive within a few months after adequate treatment. False positive VDRL - IMN and other viral infections, autoimmune diseases, and pregnancy All pregnant women should be screened early in pregnancy and at delivery. False-negative results - Antibody excess early primary syphilis latent syphilis of long duration late congenital syphilis

18. Treponemal tests - To confirm diagnosis Measure specific T. pallidum antibodies ( IgG , IgM and IgA), which appear earlier than nontreponemal ab Positive soon after initial infection Remain positive for life, Do not correlate with disease activity. T. pallidum particle agglutination test, T. pallidum hemagglutination assay, Fluorescent treponemal antibody absorption test.

19. Interpretation of nontreponemal and treponemal serologic tests in the newborn can be confounded by maternal IgG antibodies transferred to the fetus. Passively acquired antibody is suggested by a neonatal titer at least 4-fold (i.e. a 2 tube dilution) less than the maternal titer. Usually undetectable by 3-6 mo of age

20. Neurosyphilis – Difficult diagnosis Pleocytosis and increased protein in the CSF Positive CSF VDRL test (specific) + Neurologic symptoms. CSF PCR and IgM immunoblot tests also

21. Placental examination by gross and microscopic techniques can be useful in the diagnosis of congenital syphilis. The disproportionately large placentas are characterized histologically by focal proliferative villitis, endovascular and perivascular arteritis, and focal or diffuse immaturity of placental villi.

22. Diagnosis of congenital syphilis Maternal history of syphilis Proactive evaluation and treatment of exposed neonates is critical Symptomatic infants should be thoroughly evaluated and treated.

23. Diagnosis of neurosyphilis in the newborn with syphilitic infection poor sensitivity of the CSF VDRL test lack of CSF abnormalities. . Diagnosis of syphilis beyond early infancy should lead to consideration of possible child abuse. For infants with proven or highly probable disease or abnormal physical findings, complete evaluation, including serologic tests (RPR or VDRL), complete blood count with differential and platelet count, liver function tests, long-bone radiographs, ophthalmology examination, auditory brainstem response, and other tests as indicated, should be performed. For infants with a positive VDRL or RPR test result and normal physical examination whose mothers were inadequately treated, further evaluation is not necessary if 10 days of parenteral therapy are administered

24. Treatment The goals of early detection and treatment include treatment of current infection and prevention of both late stage disease and sexual or vertical transmission. T. pallidum remains extremely sensitive to penicillin, with no evidence of emerging penicillin resistance, and thus penicillin remains the treatment drug of choice (Table 245.3 and http://www.cdc.gov/std/treatment ). Parenteral penicillin G is the only documented effective treatment for congenital syphilis, syphilis during pregnancy, and neurosyphilis. Aqueous crystalline penicillin G is preferred over procaine penicillin, because it better achieves and sustains the minimum concentration of 0.018 µg/mL (0.03 units/mL) needed for 7-10 days to achieve the prolonged treponemicidal levels required for the long dividing time of T . pallidum . Although nonpenicillin regimens are available to the penicillinallergic patient, desensitization followed by standard penicillin therapy is the most reliable strategy. Success of treatment also depends upon the integrity of the host immune response. A transient acute systemic febrile reaction called the Jarisch-Herxheimer reaction (caused by massive release of endotoxin-like antigens during bacterial lysis) occurs in 15–20% of patients with acquired or congenital syphilis treated with penicillin. It is not an indication for discontinuing penicillin therapy.

25. Syphilis in Pregnancy When clinical or serologic findings suggest active infection or when diagnosis of active syphilis cannot be excluded with certainty, treatment is indicated. The goals of treatment of the pregnant woman include eradication of maternal disease, prevention of mother to child transmission, and treatment of fetal infection. Patients should be treated immediately with the penicillin regimen appropriate for the woman's stage of syphilis. Women who have been adequately treated in the past do not require additional therapy unless quantitative serology suggests evidence of reinfection (4-fold elevation in titer ). Doxycycline and tetracycline should not be administered during pregnancy, and macrolides do not effectively prevent fetal infection. Pregnant patients who are allergic to penicillin should be desensitized and treated with penicillin.

26. Adequate maternal treatment at least 30 days prior to delivery is likely to prevent congenital syphilis. All infants born to mothers with syphilis should be followed until nontreponemal serology is negative. The infant should be treated if there is any uncertainty about the adequacy of maternal treatment. The goal of infant treatment is prevention of organ damage, skeletal deformity, and developmental delay. Any infant at risk of congenital syphilis should be evaluated for HIV. Congenital syphilis is treated with aqueous penicillin G (100,000-150,000 units/kg/24 hr divided every 12 hr IV for the 1st wk of life, and every 8 hr thereafter) or procaine penicillin G (50,000 units/kg IM once daily) given for 10 days. Both penicillin regimens are recognized as adequate therapy for congenital syphilis, but higher concentrations of penicillin are achieved in the CSF of infants treated with intravenous aqueous penicillin G than in those treated with intramuscular procaine penicillin. Treated infants should be followed every 2-3 mo to confirm at least a 4-fold decrease in nontreponemal titers. Treated infants with congenital neurosyphilis should undergo clinical and CSF evaluation at 6- mo intervals until CSF is normal. At age 2 these infants should receive a full developmental assessment. In a very-low-risk neonate who is asymptomatic and whose mother was treated appropriately, without evidence of relapse or reinfection, but with a low and stable VDRL titer ( serofast ), no evaluation is necessary. Some specialists would treat such an infant with a single dose of benzathine penicillin G 50,000 units/kg IM.

27. Prevention Syphilis, including congenital syphilis, is a reportable disease in all 50 states and the District of Columbia. Testing is indicated at any time for persons with suspicious lesions, a history of recent sexual exposure to a person with syphilis, or diagnosis of another sexually transmitted infection, including HIV infection. The resurgence of syphilis compels clinicians to remain cognizant of its protean manifestations to avoid missed or late diagnosis. Timely treatment lessens risk of community spread. Despite the genome sequencing of T. pallidum in 1998, vaccine prevention remains elusive, confounded by the treponeme's ability to evade the immune system.

28. Congenital Syphilis Congenital syphilis is a preventable disease, a sentinel event indicating multiple missed opportunities. Primary prevention is tied to prevention of syphilis in women of childbearing age and secondary prevention with early diagnosis and prompt treatment of women and their partners. Access to and use of comprehensive prenatal care is key, with careful history taking (including interim sexual partners) at each visit. Routine prenatal screening for syphilis remains the most important factor in identifying infants at risk for developing congenital syphilis. Screening all women at the beginning of prenatal care is an evidence-based standard of care and legally required in all states. In pregnant women without optimal prenatal care, serologic screening for syphilis should be performed at the time pregnancy is diagnosed. Any woman who is delivered of a stillborn infant at 20 wk or fewer of gestation should be tested for syphilis.

29. In communities and populations with a high prevalence of syphilis and in patients at high risk (women with a history of incarceration, drug use, or multiple or concurrent partners), testing should be performed at least 2 additional times: at the beginning of the 3rd trimester (28 wk ) and at delivery. Some states mandate repeat testing at delivery for all women, underscoring the importance of preventive screening. Women at high risk for syphilis should be screened even more frequently, either monthly or pragmatically in the case of inconsistent prenatal care, at every medical encounter because they can have repeat infections during pregnancy or reinfection late in pregnancy. Follow-up serologic testing of all treated women should be done after treatment to document titer decline, relapse, or reinfection. No newborn should leave the hospital without the mother's syphilis status having been determined at least once during pregnancy or at delivery. In states conducting newborn screening for syphilis, both the mother's and infant's serologic results should be known before discharge. In addition, all previously uninvestigated infants of an infected mother should be screened. Strong linkages between clinicians and public health practitioners remain essential for comprehensive prevention of acquired and congenital syphilis.