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Congenital Toxoplasmosis

by Mary T. Caserta, MD

(See Toxoplasmosis.)

Congenital toxoplasmosis is caused by transplacental acquisition of Toxoplasma gondii. Manifestations, if present, are prematurity, intrauterine growth restriction, jaundice, hepatosplenomegaly, myocarditis, pneumonitis, rash, chorioretinitis, hydrocephalus, intracranial calcifications, microcephaly, and seizures. Diagnosis is by serologic testing. Treatment is with pyrimethamine, sulfadiazine, and leucovorin.

Toxoplasma gondii, a parasite found worldwide, causes congenital infection in about 1/10,000 to 80/10,000 births.

Etiology

With rare exception, congenital toxoplasmosis is due to a primary maternal infection during pregnancy. Infection with T. gondii occurs primarily from ingestion of inadequately cooked meat containing cysts or from ingestion of oocysts derived from food or water contaminated with cat feces. The rate of transmission to the fetus is higher in women infected later during pregnancy. However, women infected earlier in gestation generally have more severe disease. Overall, 30 to 40% of women infected during pregnancy will have a congenitally infected child.

Symptoms and Signs

Pregnant women infected with T. gondii generally do not have clinical manifestations, although some may have a mild mononucleosis-like syndrome, regional lymphadenopathy, or occasionally chorioretinitis. Similarly, infected neonates are usually asymptomatic at birth, but manifestations may include

  • Prematurity

  • Intrauterine growth restriction

  • Jaundice

  • Hepatosplenomegaly

  • Myocarditis

  • Pneumonitis

  • Various rashes

Neurologic involvement, often prominent, includes chorioretinitis, hydrocephalus, intracranial calcifications, microcephaly, and seizures. The classic triad of findings consists of chorioretinitis, hydrocephalus, and intracranial calcifications.

Diagnosis

  • Serial IgG measurement (for maternal infection)

  • Amniotic fluid PCR (for fetal infection)

  • Serologic testing, brain imaging, CSF analysis, ophthalmologic evaluation (for neonatal infection), and PCR testing of various body fluids or tissues

Serologic testing is important in diagnosing maternal and congenital infection. Maternal infection should be suspected if women have a mononucleosis-like syndrome and negative Epstein-Barr virus and cytomegalovirus (antibody or PCR) testing, isolated regional adenopathy not due to another cause (eg, HIV), or chorioretinitis. Acute maternal infection is suggested by seroconversion or a 4-fold rise between acute and convalescent IgG titers. However, maternal IgG antibodies may be detectable in the infant through the first year. PCR analysis of amniotic fluid is emerging as the method of choice for diagnosis of fetal infection. There are numerous other serologic tests, some of which are done only in reference laboratories. The most reliable are the Sabin-Feldman dye test, the indirect immunofluorescent antibody (IFA) test, and the direct agglutination assay. Tests to isolate the organism include inoculation into mice and tissue culture, but these tests are not usually done because they are expensive, not highly sensitive, and can take weeks before yielding results.

In suspected congenital toxoplasmosis, serologic tests, MRI or CT imaging of the brain, CSF analysis, and a thorough eye examination by an ophthalmologist should be done. CSF abnormalities include xanthochromia, pleocytosis, and increased protein concentration. The placenta is inspected for characteristic signs of T. gondii infection. Nonspecific laboratory findings include thrombocytopenia, lymphocytosis, monocytosis, eosinophilia, and elevated transaminases. PCR testing of body fluids, including CSF, and tissues can also be done to confirm infection.

Prognosis

Some children have a fulminant course with early death, whereas others have long-term neurologic sequelae. Occasionally, neurologic manifestations (eg, chorioretinitis, intellectual disability, deafness, seizures) develop years later in children who appeared normal at birth. Consequently, children with congenital toxoplasmosis should be closely monitored beyond the neonatal period.

Treatment

  • Sometimes spiramycin for pregnant women

  • Pyrimethamine, sulfadiazine, and leucovorin

Limited data suggest that treatment of infected women during pregnancy may be beneficial to the fetus. Spiramycin (available in the US with special permission from the FDA) has been used to prevent maternofetal transmission but does not provide treatment to the fetus. Pyrimethamine and sulfonamides have been used later in gestation to treat the infected fetus.

Treatment of symptomatic and asymptomatic neonates may improve outcome. Therefore, treatment is begun with pyrimethamine (initial loading dose of 2 mg/kg po once/day for 2 days followed by 1 mg/kg po once/day, maximum 25 mg) and leucovorin (10 mg po 3 times/wk). Sulfadiazine (50 mg/kg po bid, maximum 4 g) is begun after neonatal jaundice has resolved. After the initial 6 mo of treatment, sulfadiazine and leucovorin are continued at the same dose, but pyrimethamine is given less frequently (only on Monday, Wednesday, and Friday). This regimen is continued for at least 6 more mo. All treatment should be overseen by an expert. The use of corticosteroids is controversial and should be determined case by case but may be considered for active chorioretinitis or if CSF protein is >1 gm/dL.

Prevention

Pregnant women should be counseled to avoid contact with cat litter boxes and other areas contaminated with cat feces. Meat should be thoroughly cooked before consumption, and hands should be washed after handling raw meat or unwashed produce. Women at risk of primary infection (eg, those frequently exposed to cat feces) should be screened during pregnancy. Women infected during the 1st or 2nd trimester should be counseled regarding available treatments.

Key Points

  • Congenital toxoplasmosis is usually due to a primary maternal infection acquired during pregnancy; prior infection is of low risk.

  • Many organs may be affected, including heart , liver, lungs, and CNS; the classic triad of findings consists of chorioretinitis, hydrocephalus, and intracranial calcifications.

  • Some children have a fulminant course with early death, whereas others have long-term neurologic sequelae (which may not develop for several years).

  • Do PCR analysis of amniotic fluid (for fetal infection) or of body fluids (including CSF) and tissues for neonatal infection; serologic testing may also be used.

  • Do MRI or CT of the brain.

  • Pyrimethamine, sulfadiazine, and leucovorin may help.

  • Pregnant women should avoid contact with cat litter boxes and other areas contaminated with cat feces.

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