Congenital Varicella Research Articles

Update on TORCH infections in the newborn infant

TORCH infections are unique in their pathogenesis and have potentially devastating clinical manifestations. Congenital toxoplasmosis remains an important cause of blindness, although avoiding exposure to cats and uncooked meat can prevent it. Congenital syphilis has declined in incidence due to mandatory prenatal testing and effective therapy. The incidence of congenital and neonatal varicella and of congenital rubella has been lowered due to vaccination. Perinatally acquired HIV infection continues to increase at a frightening pace in the developing world. The use of antiretroviral therapy in mothers and the newborn, however, has resulted in a decrease in incidence in the United States. While cytomegalovirus remains the most common cause of congenital infection in the United States, the possibility of effective treatment with Ganciclovir (Hoffman-LaRoche, Basel, Switzerland) has emerged from recent studies. In neonatal herpes, selective use of cesarean delivery and antiviral therapy can decrease incidence and improve outcomes.

À propos de deux cas de varicelle congénitale : plaidoyer pour la vaccination des femmes en âge de procréer

We describe two cases of congenital varicella. The first presented with cutaneous aplasia and scars; the second with skin abnormalities, limb atrophy, limb paresis, Horner’s syndrome and liver calcifications: prognosis was poor in this case. After reviewing the published cases of congenital varicella, we advocate the use of varicella vaccine in seronegative women before pregnancy.

Intracerebral varicella‐zoster virus reactivation in congenital varicella syndrome

Patients with congenital varicella syndrome (CVS) typically present with clinical symptoms consisting of skin lesions, neurological defects, eye diseases, and/or limb hypoplasia. In rare cases, isolated manifestations in the brain or eye have been reported. The varicella‐zoster virus (VZV), as the causative agent of CVS, could only be detected in a few infants with CVS. In addition, there is little in the literature on antiviral treatment of infants born with signs of CVS. We report a case of CVS in a male infant who presented with generalized clonic cerebral seizures at age 4 months. An endogenous intracerebral viral reactivation following intrauterine VZV infection was assumed. After the diagnosis was confirmed virologically, acyclovir was administered intravenously for 10 days and afterwards orally for 3 weeks. This antiviral treatment was aimed at preventing progression of the disease. We concluded from this case that infants with intrauterine VZV infection can suffer intracerebral VZV reactivations that require antiviral treatment.

La varicelle au cours de la grossesse

Varicella during pregnancy is a rare occurrence because of a high rate of spontaneous immunisation in the population. When a pregnant woman presents a typical rash, maternal complications, mainly respiratory distress, can occur and be life threatening. Fetal complications include two types of consequences. Before 24 weeks of gestation, transplacental transmission is estimated around 6%, with about one third of infected fetuses presenting clinical manifestations. Congenital varicella syndrome includes growth retardation, neurological effects as microcephaly, limbs malposition, lung or bowel hyperechogenicity. But most infected fetuses will not show any anomaly. A very few might develop shingles in the first year of life. Peripartum contamination brings a much higher risk of transmission (around 25%). Neonatal varicella infection can be life threatening for the new born baby, especially if premature. A specific treatment should be started as soon as the contamination is discovered. Zoster during pregnancy does not lead to a risk of fetal infection.

La varicelle au cours de la grossesse

Varicella during pregnancy is a rare occurrence because of a high rate of spontaneous immunisation in the population. When a pregnant woman presents a typical rash, maternal complications, mainly respiratory distress, can occur and be life threatening. Fetal complications include two types of consequences. • -Before 24 weeks of gestation, transplacental transmission is estimated around 6 %, with about 1/3 of infected fetuses presenting clinical manifestations. Congenital varicella syndrome includes growth retardation, neurological effects as microcephaly, limbs malposition, lung or bowel hyperechogenicity. But 2/3 infected fetuses will not show any anomaly. A very few might develop shingles in the first year of life. • -Peripartum contamination brings a much higher risk of transmission (around 25 %). Neonatal varicella infection can be life threatening for the new born baby, specially if premature. A specific treatment should be started as soon as the contamination is discovered. Zoster during pregnancy does not lead to a risk of fetal infection.

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.

Frequency of congenital varicella syndrome in a prospective cohort of 347 pregnant women

OBJECTIVE:To estimate the rate of congenital varicella zoster virus syndrome in neonates born to women developing varicella zoster virus infections during pregnancy.METHODS:Pregnant women with clinical varicella zoster virus infection were enrolled at ten perinatal centers. Maternal and fetal immunoglobulin (Ig) G and IgM by fluorescent antibody confirmed 74.3% of cases. Specialists examined neonates at 0–6 months, 7–18 months, and 19–30 months after delivery to detect abnormalities of their eyes, hearing, and physical and developmental features. A hierarchical set of criteria was used to define congenital varicella syndrome. A jury of four investigators assigned the classification of all findings.RESULTS:In 362 women enrolled from 1993 to 1996, 15 had herpes zoster, and 347 had primary varicella zoster virus infection. Varicella zoster virus affected 140 women (38.7%) in the first trimester, 122 (33.7%) in the second trimester, and 100 (27.6%) in the third trimester. Five twin pairs were included. Only one case (0.4%) of definite congenital varicella syndrome was found, a 3360-g female infant having a left retinal macular lesion with typical skin scars after maternal varicella at 24 weeks. The maternal blood sample at birth was negative for IgG antibodies to toxoplasmosis and cytomegalovirus. Two cases involved fetal death at 20 weeks and fetal hydrops at 17 weeks after maternal varicella at 11 and 5 weeks, respectively. We found no cases of limb hypoplasia, microcephalus, or cataract.CONCLUSION:The frequency of congenital varicella syndrome is very low (0.4%) in a prospectively studied cohort. Eye examinations of exposed infants had a low yield.

Frequency of Congenital Varicella Syndrome in a Prospective Cohort of 347 Pregnant Women

Frequency of Congenital Varicella Syndrome in a Prospective Cohort of 347 Pregnant Women

Varicella infection in pregnancy.

Varicella (chickenpox) is a common childhood illness. Most adults are immune to the virus because of previous exposure. Pregnant women who contract varicella risk complications such as pneumonia. Varicella may be transmitted from mother to fetus and could cause congenital varicella syndrome or perinatal infection. Susceptibility to varicella should be determined before pregnancy. Varicella zoster immune globulin may be considered for the mother or newborn if exposure occurs. Acyclovir may decrease the risk of maternal complications from infection.

Congenital varicella syndrome as an unusual cause of congenital malformation: report of one case.

Intrauterine infections with varicella-zoster virus following maternal varicella in early pregnancy and resulting in congenital malformations are rare. Herein we report a child with congenital varicella syndrome characterized by low birth weight, cicatricial scarring, hypoplasia of both lower extremities with joint contracture, congenital hip dislocation, corneal opacity, atresia of the sigmoid colon and a rarely associated cloaca anomaly. The varicella IgG remained positive after she was seven months old. Her mother developed chickenpox at the 14th week of gestation. The purpose of this article is to raise pediatricians' index of suspicion for congenital varicella syndrome when an infant is born with multiple congenital malformations with an apparent history of maternal varicella infection.

Varicella vaccine exposure during pregnancy: data from the first 5 years of the pregnancy registry

Objective: To assess the risks of congenital varicella syndrome and other birth defects in offspring of women who inadvertently received varicella vaccine during pregnancy or within 3 months of conception. Methods: Pregnant women inadvertently exposed to varicella vaccine, reported voluntarily, were enrolled in the Pregnancy Registry for VARIVAX (Merck & Co., Inc., West Point, PA). The pregnancies were monitored and the outcomes ascertained from questionnaires completed voluntarily by the health care providers. The rates of congenital varicella syndrome and congenital anomalies were calculated for seronegative women prospectively reported to the registry. Results: From March 17, 1995 through March 16, 2000, 362 pregnancy outcomes were identified from prospective reports. Ninety-two women were known to be seronegative to varicella, of whom 58 received their first dose of vaccine during the first or second trimester. No cases of congenital varicella syndrome were identified among 56 live births (rate 0%, 95% confidence interval [CI] 0, 15.6). Among all the prospective reports of live births, five congenital anomalies were reported. No specific pattern was identified in either the susceptible cohort or the sample population as a whole. Conclusion: No abnormal features have been reported that suggested the occurrence of congenital varicella syndrome or other birth defects related to vaccine exposure during pregnancy. Because of the small numbers, this study has limited precision, so continued surveillance is warranted. However, these results should provide some assurance to health care providers and women with inadvertent exposure before or during pregnancy.

The management of varicella-zoster virus exposure and infection in pregnancy and the newborn period. Australasian Subgroup in Paediatric Infectious Diseases of the Australasian Society for Infectious Diseases.

Zoster immunoglobulin (ZIG) should be offered to pregnant, varicella-seronegative women with significant exposure to varicella-zoster virus (VZV) (chickenpox) infection. Oral aciclovir prophylaxis should be considered for susceptible pregnant women exposed to VZV who did not receive ZIG or have risk factors for severe disease. Intravenous aciclovir should be given to pregnant women who develop complicated varicella at any stage of pregnancy. Counselling on the risk of congenital varicella syndrome is recommended for pregnant women who develop chickenpox. ZIG should be given to a baby whose mother develops chickenpox up to 7 days before delivery or up to 28 days after delivery. Intravenous aciclovir should be given to babies presenting unwell with chickenpox, whether or not they received ZIG. Breastfeeding of babies infected with or exposed to VZV is encouraged. A mother with chickenpox or zoster does not need to be isolated from her own baby. If siblings at home have chickenpox, a newborn baby should be given ZIG if its mother is seronegative. The newborn baby does not need to be isolated from its siblings with chickenpox, whether or not the baby was given ZIG. After significant nursery exposure to VZV, ZIG should be given to seronegative babies and to all babies born before 28 weeks' gestation.

Severe Complications Associated with Varicella: Province of Quebec, April 1994 to March 1996

To determine the frequency and severity of serious complications associated with varicella in Quebec; the frequency and severity of cases of congenital varicella; and hospital costs associated with hospitalizations for varicella. All hospitalizations related to varicella were identified through the use of a hospital data bank and pertinent data were collected from hospital records. Province of Quebec with a population of 6,895,960 people. All cases with a principal or secondary diagnosis of varicella hospitalized in Quebec between April 1, 1994 and March 31, 1996. Types of complications and reason for hospitalization, risk of complications and calculation of associated costs were studied. Nine hundred nine eligible hospitalizations were identified between April 1, 1994 and March 31, 1996. In all, 583 (64.1%) hospitalizations were for the treatment of complications, 127 (14.0%) for administration of intravenous acyclovir and 199 (21.9%) for supportive care. Healthy people accounted for 644 (70.8%) hospitalizations and immunosuppressed individuals for 136 (15.0%). Among children, one-half of the principal complications were skin infections, while 13.5% and 8.4% of principal complications were pneumonia and neurological complications, respectively. Among adults, the most common complication was pneumonia, with a rate of 43.5%, followed by thrombocytopenia and skin infections, with rates of 22.2% and 14.8%, respectively. The complication rate was 29.2 cases/10,000 cases of varicella. Although perceived as a benign childhood disease by the general population, varicella may be accompanied by severe complications. Morbidity associated with varicella is one of the elements that must be considered when evaluating the usefulness of varicella vaccine.

The congenital varicella syndrome.

Maternal varicella during the first two trimesters of pregnancy may cause the congenital varicella syndrome (CVS). After infection in the first 20 weeks' gestation, the incidence is estimated to be about 2%. To date, nearly 100 infants born with signs of CVS have been reported in the literature, more than three quarters of them during the last two decades. The characteristic symptoms consist of skin lesions in dermatomal distribution (76%), neurologic defects (60%), eye diseases (51%), and skeletal anomalies (49%). About 30% of infants born with these lesions died in the first months of life. The diagnosis of CVS should be established by the appearance of maternal varicella, the presence of typical clinical symptoms as well as the laboratory evidence of in utero varicella-zoster virus (VZV) infection. In the reviewed infants, intrauterine VZV-infection has been proved in about 60%. Passive immunization may reduce the risk of fetal infection but there is no evidence to prevent fetal viremia. Up to now, there are no controlled studies concerning antiviral chemotherapy in preventing CVS.

Ipsilateral renal dysplasia with hypertensive heart disease in an infant with cutaneous varicella lesions: An unusual presentation of congenital varicella syndrome

A child with congenital varicella syndrome including cutaneous lesions and ipsilateral renal dysplasia with hypertensive heart disease is described. Varicella was contracted during the tenth week of gestation. Typical congenital varicella bullae, high titer of anti-varicella-zoster virus IgM, and a small right kidney were noted after birth. Hypertensive heart disease resulting from renal dysplasia occurred at 1 year of age. The cutaneous lesions and the dysplastic kidney involved the same dermatomes. Nephrectomy proved to be the treatment of choice for hypertension and congestive heart failure. (J Am Acad Dermatol 2000;43:864-6.)

Congenital and neonatal varicella in Australia

To establish the incidence and severity of congenital and neonatal varicella in Australia. Demographic and clinical details were obtained by postal questionnaire regarding cases notified to the Australian Paediatric Surveillance Unit by over 930 participating clinicians in 1995-97 inclusive. Seven cases of congenital varicella (1: 107 000 pregnancies/year) followed maternal infection at 8-26 weeks: five had defects, two did not. Four of the seven infants with congenital varicella developed herpes zoster in the first 15 months of life. Forty-four infants had neonatal varicella (1: 17 000 pregnancies/year). : There is an ongoing, albeit low, incidence of congenital and neonatal varicella in Australia.

Viral diseases in pregnancy: a review of rubella, chickenpox, measles, mumps and 5th disease

Approximately 10–15% of women of reproductive age are susceptible to Rubella. Such patients should be vaccinated immediately postpartum to prevent concern about congenital infection in future pregnancies. More than 90% of women of reproductive age are immune to varicella. Susceptible patients who are exposed to varicella should receive immunoprophylaxis with varicella zoster immune globulin. When mothers acquire chickenpox in the first 20 weeks of pregnancy, the overall risk of congenital varicella infection is ≤2%. Measles infection during pregnancy is not associated with an increased incidence of fetal malformations. However, infected mothers are at risk for serious complications such as otitis media, encephalitis, and pneumonia. Mumps infection during pregnancy rarely poses a serious risk of maternal or fetal morbidity. In contrast, maternal parvovirus infection during the first 20 weeks of gestation may be associated with a 5–10% risk of fetal hydrops. This complication can be treated successfully with intrauterine transfusion.

Obstetric Brachial Plexus Palsy

Objectives: to describe my research into and conclusions about obstetric brachial plexus palsy with special emphasis on aspects that would be of interest to obstetricians.Materials and Methods: based on an extensive literature review as well as on the research I was involved in concerning birth palsy, the following points are investigated: classification of the types of traumatic birth palsy, risk factors, congenital and intra-uterine palsies, evaluation, prognostic factors for spontaneous recovery and surgery for obstetric paralysis.Results: infants with birth palsy should be assessed by a multidisciplinary team. Traumatic brachial plexus injury is classified into four types: Erb’s, total, Klumpke’s and intermediate palsies. Each type is associated with a specific intrapartum force and each has a specific clinical presentation. Risk factors for birth palsy include fetal macrosomia, shoulder dystocia, maternal short stature, gestational diabetes, vaginal breech delivery, instrumental delivery and a previous history of giving birth to a baby with birth palsy. The hand surgeon is frequently involved in the medico-legal assessment of birth palsy cases and, hence, it is important to realize that paralysis of the limb in the newborn is not always due to traumatic delivery. Other causes of paralysis include: congenital aplasia of the roots of the brachial plexus, congenital varicella, cerebral palsy, intra-uterine nerve compression by the umbilical cord or amniotic bands, “Deformation Syndrome“ and “intra-uterine mal-adaptation palsy”. Infants with birth palsy should be evaluated at regular intervals by the hand surgeon and have continuous physiotherapy exercises. The most important factor that indicates good spontaneous recovery is early clinical improvement of limb function. Infants with poor spontaneous recovery will benefit from surgical exploration and reconstruction of the brachial plexus.Conclusion: obstetric brachial plexus palsy has become a subject of interest in the surgical literature because of advances in microsurgery and nerve reconstruction. This has led to several published papers on the topic dealing with the re-classification of the types of palsy, its risk factors, aetiology, evaluation, prognosis and new concepts of surgical intervention.

Kongenitale Langerhans-Zellen-Histiozytose unter dem klinischen Bild einer Varizelleninfektion

We report on a preterm infant (33rd gestational week) with a varicella-like congenital rash, which initially appeared to respond to therapy with acyclovir. At the age of 3 weeks, lesions were in different stages of evolution and still resembled a varicella zoster virus (VZV) infection. However, since proof of VZV infection was lacking and new lesions erupted at the age of 4 weeks, a skin biopsy was performed which revealed a diagnosis of Langerhans cells histiocytosis. Therapy with prednisone resulted in prompt healing of the lesions. Congenital Langerhans cell histiocytosis is rare and symptoms may vary substantially from case to case. Like in our observation it may be confused with congenital varicella. In case of congenital skin lesions of uncertain etiology a skin biopsy should be performed.

Varicella-zoster virus infection in Australia.

To determine the epidemiology of varicella-zoster virus (VZV) infection in Australia using currently available data sources. Analysis of national death data (23 years), congenital and neonatal cases (one year) and attendances at sentinel general practices (two years); hospital admissions in NSW and SA (six years); serological studies in 1995 involving antenatal clinics in Sydney and Brisbane and child-care centre staff and refugees in Sydney; and case-ascertainment in 1995 in South Western Sydney among public hospital staff, child-care centre staff and the community. In Australia, there have been an average of 3.5 deaths from chickenpox (mostly children) and 11 from herpes zoster (mostly older people) each year since 1980. The crude death rate for chickenpox has declined (p > 0.05). In 1995, there were 14 cases of neonatal and two of congenital varicella. Average annual admission rates for NSW and SA showed 1,200 hospital bed-days used for chickenpox, more than 20% with complications, and more than 7,300 bed days for zoster; annually more than 880 in-patient admissions were complicated by VZV. Most people encounter the virus in their first 15 years, but some remain susceptible into their 20s; 25% of cases and 37% of hospital admissions for chickenpox occur in people > or = 15 years of age. VZV infection involves people of all ages. It causes substantial morbidity and mortality, particularly at the extremes of life. The death rate from chickenpox but not zoster has fallen since the introduction of acyclovir in the 1980s. Surveillance of VZV infection must be given priority once vaccines become available, to monitor changes in morbidity and mortality.