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  1. Definition. Syphilis is a sexually transmitted disease caused by Treponema pallidum.Early congenital syphilis (CS) is when clinical manifestations occur at <2 years of age; late congenital syphilis is when manifestations occur at >2 years of age. In 1990, a new surveillance case definition for congenital syphilis was adopted by the Centers for Disease Control and Prevention (CDC) to improve reporting of congenital syphilis by public health agencies. It calls for reporting all infants (and stillbirths) born to women with untreated or inadequately treated syphilis at delivery, regardless of neonatal symptoms or findings.

  2. Incidence. The incidence of CS parallels that of primary and secondary syphilis in the general population. The most recent incidence in the United States is 8.8 cases per 100,000 live births. Worldwide, syphilis continues to represent a serious public health problem especially in developing countries. The World Health Organization estimates that a million pregnancies a year are adversely affected by maternal syphilis with 460,000 such pregnancies resulting in abortion or perinatal death and 270,000 infants born with CS.

  3. Pathophysiology. Treponemes appear able to cross the placenta at any time during pregnancy, thereby infecting the fetus. Syphilis can cause preterm delivery, stillbirth (30–40% of fetuses with CS are stillborn), congenital infection, or neonatal death, depending on the stage of maternal infection and duration of fetal infection before delivery. Untreated infection in the first and second trimesters often leads to significant fetal morbidity, whereas with third-trimester infection many infants are asymptomatic. The most common cause of fetal death is placental infection associated with decreasing blood flow to the fetus, although direct fetal infection also plays a role. Infection can also be acquired by the neonate via contact with infectious lesions during passage through the birth canal. Kassowitz law states that the risk of vertical transmission of syphilis from an infected, untreated mother decreases as maternal disease progresses. Thus transmission ranges from 70–100% in primary syphilis, 40% for early latent syphilis to 10% for late latent disease. CS can cause placentomegaly and congenital hydrops.

  4. Risk factors. Infants whose mothers received no or inadequate treatment (dose was unknown, inadequate, or undocumented), the mother received a nonpenicillin treatment during pregnancy for syphilis, or the mother was treated within 28 days of the infant's birth. Infants of high-risk mothers (drug use, especially cocaine use, low socioeconomic levels, HIV infection, teen pregnancy, lack of prenatal care) are at increased risk for syphilis. Lack of prenatal care is the strongest predictor of CS.

  5. Clinical presentation. CS is a multiorgan infection that may cause neurologic or skeletal disabilities or death in the fetus or newborn. However, when mothers with syphilis are treated early in pregnancy, the disease is almost entirely preventable. Spirochetes can cross the placenta and infect the fetus from ~14 weeks' gestation, with the risk of fetal infection increasing with advancing gestation. About two thirds of liveborn neonates with CS are asymptomatic at birth but have low birthweight. Clinical manifestations after birth are arbitrarily divided into early CS (<2 years of age) and late CS (>2 years old).

      1. Early manifestations include ...

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