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Congenital Syphilis
Dr Ira Shah
M.D, DNB, DCH(Gold Medalist), FCPS

Congenital syphilis occurs due to transplacental transmission of spirochetes. Untreated pregnant women with primary and secondary syphilis and spirochetemia are more likely to transmit infection than women with latent infection. Transmission can occur throughout pregnancy and 40% of affected fetuses die in intrauterine or perinatal period. Clinical manifestations of congenital infections are varied and involve multiple organ systems. Majority of infants are asymptomatic at time of birth, but if untreated symptoms develop within weeks or months. Early congenital syphilis is analogous to secondary stage of acquired syphilis. Patients may present with hepatosplenomegaly, jaundice and elevated liver transaminases. Lymphadenopathy may be present. Coomb negative hemolytic anemia may be seen. Thrombocytopenia due to platelet trapping in spleen is common. The characteristic rash seen is mucocutaneous erythematous maculopapular lesions followed by desquamation over hands and feet as seen in our patient. Rhinitis and condylomatous lesions over mucus membranes are common.

Bone involvement in form of painful osteochondritis at wrists, elbows ankles and knees occur frequently. Periostitis of long bones may be seen. CNS involvement, failure to thrive, chorioretinitis and nephrotic syndrome may also be seen.

Late congenital syphilis results primarily from chronic inflammation of bone, teeth and CNS. Bony prominence of forehead (olympian brow), thickening of sternoclavicular portion of the clavicle, anterior bowing of the mild portion of tibia (saber shins) and scaphoid scapula result due to persistent or recurrent periostitis. Peg shaped upper central permanent incisors and notched enamel (Hutchison teeth) are common. Saddle nose due to syphilitic rhinitis and a perforated nasal septum can occur. Other features of late congenital syphilis are Juvenile paresis, juvenile tabes dorsalis, aortitis, interstitial keratitis, eight nerve deafness and clutton’s joints.

Diagnosis of congenital syphilis is made when the mother of an infant has a reactive treponemal and non-treponemal serologic test with characteristic clinical findings in the infant or when T. pallidum is demonstrated by dark-field microscopy or immunoflorescence from placental, skin or umbilical lesions.

Non-treponemal test such as Venereal Disease Research Laboratory (VDRL) is useful for correlating disease activity. Titers rise when disease is active and fall when treatment is adequate. VDRL usually becomes non-reactive in 1 year for treated primary syphilis and in 2 years for treated secondary syphilis. False positive VDRL can occur in uninfected infant due to passively transferred maternal antibody and is suggested when neonatal titers are less than maternal titers. These passively transferred antibodies disappear by 3 months of age.

Treponemal tests such as florescent treponemal antibody absorption test (FTA-ABS) are used for confirmation of positive results of VDRL. These tests remain positive for life, even when the patient is treated and hence cannot be used for monitoring treatment.

Treatment

CDC recommends treatment for following infants:

Born to mothers who had untreated syphilis at birth
Evidence of maternal relapse or reinfection
Evidence of active disease in infant
Radiologic evidence of syphilis
Reactive CSF VDRL in infants
Infant’s VDRL titre at least fourfold greater than the mother’s titre.

If maternal treatment is adequate and more than 1 month below delivery and if infant’s VDRL test represents passively transferred antibody, the infant does not treatment and follow up VDRL is recommended.

Since CSF VDRL is not very sensitive, all infants with diagnosis of congenital syphilis should be treated with regimen for neurosyphilis.

Treatment consists of crystalline penicillin (1,00,000-1,50,000 U/kg/day bd for 7 days & thereafter 8 hourly for 10-14 days) or procaine penicillin (50,000 U/kg/m daily for 10-14 days). Treated infants should be followed serologically to confirm decreasing VDRL titers

References
  1. Behram RE, Kliegman RM, Jenson HB. In: Nelson’s Textbook of Pediatrics, 17th ed. Philadelphia, W.B. Saunders, 2004
Last Updated on 11-08-2007

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