Cases reported "Fetal Diseases"

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1/14. prenatal diagnosis of congenital varicella syndrome and detection of varicella-zoster virus in the fetus: a case report.

    Varicella syndrome (VS) specific malformations were sonographically seen at 22 weeks and 3 days of gestation. Fetal infection was demonstrated by detection of varicella-zoster virus (VZV) dna in fetal blood and amniotic fluid by polymerase chain reaction (PCR). Following therapeutic abortion, fetal infection was confirmed by detection of VZV dna in several fetal tissues and placenta, and by histopathological findings like miliary calcified necroses in fetal organs.
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2/14. Fetal varicella syndrome: disruption of neural development and persistent inflammation of non-neural tissues.

    Primary varicella zoster virus (VZV) infection during pregnancy is rare. If it occurs between the 8th and 20th week of gestation, fetal varicella syndrome results in 1-2% of the fetuses. We report about a varicella infection that affected a pregnant mother in the 12th week of gestation. At 33 weeks, a premature girl was born with destruction of neurons in spinal cord, spinal ganglia and plexus myentericus, and secondary developmental disturbance including mummification of one arm and segmental intestinal atresia. The brain did not show any abnormalities. However, VZV dna could be detected by PCR in tissues from the brain and spinal ganglia. Chronic necrotizing inflammation was found in the placenta, fetal membranes, and one ovary. These locations showed nuclear inclusions which by in-situ-hybridization were proven to be VZV derived. This case demonstrates that in the fetal age, 'neurotropism' of VZV signifies severe destruction but not necessarily persistent inflammation of neural tissue. However, due to the inefficient fetal immune system, inflammation can go on for weeks, preferentially in non-neural tissues.
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3/14. The natural history of varicella embryopathy: a 25-year follow-up.

    A patient with clinically and immunologically proven varicella embryopathy achieved substantial recovery after initial severe developmental delay and manages well with her residual physical disabilities in adulthood.
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4/14. Fetal varicella-herpes zoster syndrome in early pregnancy: ultrasonographic and morphological correlation.

    We report a case of an intrauterine fetal infection by the varicella-herpes zoster virus following maternal varicella at 17 weeks' amenorrhea. prenatal diagnosis of fetal infection was confirmed by serology and fetal damage by ultrasonography. autopsy of the fetus showed multiorgan manifestation with disseminated foci of necrosis and microcalcifications, encephalitis and unilateral segmental skin scarring with an underlying hypoplastic fixed lower limb. The placenta showed a multifocal chronic villitis with multinucleated giant cells. The lesions included segmental anomalies and multiorgan damage.
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5/14. Congenital varicella syndrome: case with isolated brain damage.

    We report a case of congenital varicella syndrome after maternal infection during the 10th week of pregnancy. None of the typical abnormalities were manifested at birth; however, the child experienced neonatal tonic convulsions that evolved to refractory focal epilepsy with adversive, complex partial, and secondarily generalized seizures. At the age of 5 years, after a prolonged generalized seizure following cranial trauma, cranial computed tomography (CT) was performed. It led to the misdiagnosis of post-traumatic intracerebral hemorrhage. Afterwards, because of persistent seizures and developmental delay with mild or slight intellectual deficit, the next CT and magnetic resonance imaging (MRI) investigation at 8 years of age disclosed massive malformation of the left cerebral hemisphere. The serologic evidence of varicella-zoster virus-specific IgG antibodies without a history of varicella disease after birth and maternal infection during early pregnancy were crucial to diagnosing the rare syndrome of congenital varicella consisting of isolated brain damage.
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6/14. Congenital varicella syndrome.

    The fetal consequences of chickenpox complicating pregnancy depends on the period of gestation at which the infection is contracted. The extremely rare classical form of congenital varicella syndrome, resulting from maternal varicella infection in the first trimester of pregnancy, is being reported here. The unusual features in this baby are bilateral hypoplastic lower extremities, fracture of bones, a normal electroencephalogram and phantom hernias of the anterior abdominal wall.
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7/14. Prenatal ultrasound and magnetic resonance imaging in fetal varicella syndrome: correlation with pathology findings.

    OBJECTIVES: To assess the diagnostic value of prenatal magnetic resonance imaging (MRI) in addition to prenatal ultrasound in a case of fetal varicella syndrome. methods: Comparison of prenatal ultrasound and MRI features obtained at 26 and 32 weeks, respectively, with neonatal imaging (ultrasound, MRI and CT) and macroscopic and microscopic pathology findings in a fatal case of varicella embryopathy. RESULTS: Prenatal ultrasound correlated fairly well with neonatal imaging and pathology findings. Most lesions of thoracic, abdominal and retroperitoneal viscera, limb involvement and even dermatologic features were apparent on ultrasonography. Involvement of the CNS, including cerebellar hypoplasia, was not apparent on ultrasound examination, but was clearly demonstrated by prenatal MRI. CONCLUSION: If maternal seroconversion for the varicella-zoster virus is suspected, combining prenatal ultrasound and magnetic resonance imaging may document the extent of tissue damage in fetal varicella syndrome to a larger extent than has been reported until now and therefore contribute to due counselling following maternal varicella exposure.
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8/14. Distribution of varicella-zoster virus dna and gene products in tissues of a first-trimester varicella-infected fetus.

    Precise information about varicella-zoster virus (VZV) infection in first-trimester fetuses remains sketchy. After varicella infection was diagnosed in a woman, her 12-week-old fetus was aborted and was investigated, by histological examination, virus culturing, polymerase chain reaction, in situ hybridization (ISH), and immunohistochemistry (IHC), for the presence of VZV infection. Only the results of the histological examination suggested the presence of alpha -herpesvirus infection, in the gastrointestinal tract and liver; results of ISH were positive for VZV, and results of IHC staining were positive for intermediate early protein 63 (IE63) but negative for glycoprotein E (gE), in the dorsal root ganglia (DRG), meninges, gastrointestinal tract, pancreas, smooth muscle, liver, and placental trophoblast, indicating the presence of a nonproductive, latency-like VZV infection. Only the gastrointestinal tract and liver exhibited simultaneous staining for IE63 and gE, a result suggesting that active replication of VZV was present. In conclusion, widespread nonproductive VZV infection in the absence of histological clues is an early event in VZV infection in fetuses. The observed gene-expression pattern in most tissues resembles that of latent VZV infection in DRG. Latency-like infection in nonneural cell types may potentially reactivate, leading to multifocal necrosis, fibrosis, and dystrophic calcifications, as observed in advanced congenital varicella syndrome.
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9/14. Prenatal ultrasound diagnosis, follow-up, and outcome of congenital varicella syndrome.

    OBJECTIVES: To report on a case of fetal varicella infection following the diagnosis of maternal infection at 16 weeks of gestation. methods: Diagnosis was based on serology testing and prenatal ultrasound, confirmed by dna detection in amniotic fluid (Lightcycler-PCR). Serial ultrasound examinations were performed. RESULTS: Sonographic anomalies included borderline ventriculomegaly, intracerebral, intrahepatic and myocardial calcifications, limb deformities, articular effusions, and intrauterine growth retardation (confirmed postpartally). The newborn showed a severe encephalopathy and could not be stabilized sufficiently. The child died 23 days after birth. CONCLUSION: The outcome of an affected fetus may be very serious and prenatal ultrasound is a helpful tool to recognize the severity of the infection.
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10/14. In utero diagnosis of congenital varicella zoster virus infection by chorionic villus sampling and polymerase chain reaction.

    Varicella zoster virus infection acquired in pregnancy is reported to cause fetal damage in 5% to 10% of cases. We used polymerase chain reaction to attempt molecular diagnosis of fetoplacental varicella zoster virus infection in two patients. Tissue obtained by chorionic villus sampling in the second trimester was analyzed by polymerase chain reaction with a varicella zoster virus-specific primer, ORF-63, and was found to be positive in both patients. Viral cultures were negative. One patient elected pregnancy termination at 23 weeks. Southern blot hybridization of neonatal brain tissue for varicella zoster virus was negative. The second patient carried the pregnancy to term and was delivered of a normal infant. Varicella zoster virus immunoglobulin m and viral cultures were negative. The presence of viral deoxyribonucleic acid sequences in placental tissue does not correlate with fetal disease.
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