CONGENITAL SYPHILIS
Dr Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai, India
Address for Correspondence
Dr Ira Shah, 1/B Saguna, 271/B St. Francis Road, Vile Parle (W), Mumbai 400056.
Abstract
Inspite of adequate antenatal screening, cases of congenital syphilis still arise. We present a child with congenital syphilis who was diagnosed at 7 months of age due to characteristic skin lesions. The mother was diagnosed to be VDRL positive during pregnancy, however, the child was not investigated for same after birth and was subsequently diagnosed only much later in life.
Introduction
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 (1). 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 (2). We present a child with congenital syphilis who was asymptomatic at birth who was diagnosed at 7 months of age due to characteristic skin lesions. The mother was diagnosed to be VDRL positive during pregnancy, however, the child was not investigated for same after birth.
Case Report
A 7 month old boy born of non consanguineous marriage presented with vomiting and diarrhea since 15 days. He was full term normal delivery with birth weight of 2.5 kg. Milestones and immunization were fill date. On examination, he was malnourished [Height = 50 cm, <5 th centile; Weight = 6 kg, <5 th centile] and severely dehydrated. His anterior fontanelle was open 5 cm x 5 cm, and he had peeling of skin of palms and soles (Figure 1). He had bilateral purulent eye discharge. Other systemic examination was normal. In view of the characteristic skin lesions, he was suspected as a case of congenital syphilis. The mother was asked to bring back her antenatal card which showed a positive VDRL in the mother (1:32 titre) in 6th month of gestation. Mother had been treated with Inj Benzathine Penicillin. The child's VDRL showed high titres of 1:1240 and CSF VDRL was negative. Simultaneous, mother's VDRL was 1:16. His HIV, HBsAg and Anti HCV were negative. Other investigations revealed normal hemogram, normal liver and renal function tests, normal x-ray chest and normal CSF. The infant was treated with Inj Crystalline Penicillin for 21 days following which his VDRL became non-reactive. He was advised regular follow up.

Figure 1: Typical rash of congenital syphilis
Discussion
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. (1) 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. (1) 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 (1).

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. (3)
Conclusion
CDC recommends treatment for following infants: 1) Born to mothers who had untreated syphilis at birth, 2) Evidence of maternal relapse or reinfection, 3) Evidence of active disease in infant, 4) Radiologic evidence of syphilis, 5) Reactive CSF VDRL in infants, 6) 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 (4). 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 (4).
References :
  1. Feigin RD, Cherry JD. Syphilis. In: Textbook of Pediatric Infectious Diseases. 1998:1543-56
  2. American Academy of Pediatrics. Syphillis. In: Red Book: Report of the Committee on Infectious Diseases. 26th ed. 2003:595-607
  3. Behram RE, Kliegman RM, Jenson HB. In: Nelson's Textbook of Pediatrics, 17th ed. Philadelphia, W.B. Saunders, 2004
  4. CDC. Sexually transmitted diseases treatment guidelines, 2002. MMWR 2002;51(No. RR-6)
Last Updated : Monday, August 01, 2005 Vol 2 Issue 8 Art #39
How to Cite URL :
Shah I. CONGENITAL SYPHILIS. Pediatric Oncall [serial online] 2005[cited 2005 August 1];2. Art #39. Available From : http://www.pediatriconcall.com/Journal/Article/FullText.aspx?artid=731&type=J&tid=&imgid=&reportid=181&tbltype=
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