Unusual cause of brachial palsy with diaphragmatic palsy.

We report a preterm neonate born with respiratory distress. The neonate was found to have diaphragmatic palsy and brachial palsy. The neonate was born by caesarean section and there was no history of birth trauma. On examination, there was bilateral congenital talipes equinovarus and a scar was present on the forearm. The mother had a history of chickenpox during the 16 weeks of pregnancy for which no treatment was sought. On investigation, PCR for varicella was found to be positive in the neonate.

Isolated lower limb hypoplasia secondary to congenital varicella syndrome: a rare occurrence and management of its complications.

Isolated lower limb hypoplasia is a rare consequence of maternal congenital varicella syndrome (CVS). The hypoplastic limb is susceptible to multiple injuries, including fractures, especially if there is associated muscle weakness and lack of sensation. We describe a unique index case of a woman aged 26 years with a background of CVS who presented with a distal femur fracture following a fall onto her insensate, hypoplastic right leg. This report highlights the complexities involved in the diagnosis and management of fractures in patients with an anaesthetic limb, and in particular describes limb amputation as a successful treatment modality for distal femur fractures.

Skin infections in pregnancy.

A wide array of infectious diseases can occur in pregnancy. Their acquisition, clinical presentation, and course during gestation may be altered due to an impairment of the maternal cellular immunity. Some infectious diseases can lead to serious consequences for the mother or the offspring, including congenital malformations. This review describes in detail the clinical presentation, course, management, and associated maternal and fetal risks of selected viral (varicella-zoster virus infections, condylomata acuminata), fungal (candida vulvovaginitis), bacterial (Lyme borreliosis), and parasitic (scabies) infections. The treatment options are critically reviewed. First-line therapies include acyclovir and varicella-zoster virus immunoglobulin for varicella-zoster virus infections, surgical modalities for genital warts, topical clotrimazole and oral fluconazole for Candida vulvovaginitis, amoxicillin and cefuroxime for Lyme borreliosis, and permethrin for scabies. A synopsis of maternal and fetal risks of other important infections is also included.

Congenital varicella syndrome: A systematic review.

Varicella-zoster virus (VZV) is a teratogen that can cross the placenta and cause the congenital varicella syndrome (CVS), which is characterised by multi-system anomalies. There have been 130 reported cases of CVS from 1947 to 2013. The estimated incidence of CVS was 0.59% and 0.84% for women infected with VZV during the entire pregnancy and for those infected the first 20 weeks of pregnancy, respectively. Nine cases were reported at 21-27 weeks of gestation and one case was identified at 36 weeks. Herpes zoster caused CVS in two cases. Regarding treatment, varicella zoster immunoglobulin treatment, irrespective of gestational age, should be considered in addition to antiviral drugs for women who have been exposed to or infected with virus.

Síndrome de varicela congénita / Congenital varicella syndrome

Se estima que la transmisión vertical del virus de la varicela ocurre entre un 8 y un 25% de los casos. De estos, solo en un 1-2% de las ocasiones se producirá el síndrome de varicela congénita (SVC), sobre todo si la infección ocurre entre las semanas 12-20 de gestación. La detección del DNA del virus en líquido amniótico, junto con la presencia de marcadores ecográficos de afectación fetal, hacen el diagnóstico altamente probable. Presentamos el caso de una tercigesta que en la semana 14.ª de gestación contrajo varicela, ocurriendo afectación fetal (calcificaciones hepáticas y miocárdicas en ecografía) y muerte del neonato al mes de vida. Actualmente, la inmunización pasiva representa la única estrategia activa para prevenir las graves consecuencias del SVC si una gestante no inmune se expone al VVZ antes de la semana 20 de gestación, ya que, tanto el uso de antivirales como profilaxis o como tratamiento como el uso de inmunoglobulina anti VVZ tras un contacto, no han mostrado resultados concluyentes. Por todo esto, consideramos capital la correcta información a los padres y el adecuado control de este tipo de gestaciones (AU)

Vertical transmission of the varicella virus is estimated to occur in 8% to 25% of cases. Among these, congenital varicella syndrome develops in only 1% or 2% of transmissions, especially if the infection occurs between weeks 12 and 20 of pregnancy. The detection of DNA from the virus in the amniotic fluid, combined with the presence of ultrasonographic markers of fetal involvement, leads to a highly likely diagnosis. We present the case of a gravida 3 who contracted varicella at week 14 of gestation, with fetal involvement (hepatic and myocardial calcifications detected on ultrasonography) and newborn death at 1 month of life. Currently, passive immunization is the only active strategy to prevent the serious consequences of congenital varicella syndrome if a non-immune pregnant woman is exposed to the varicella zoster virus before week 20 of pregnancy, since neither the use of antiviral prophylaxis or treatment, nor the use of anti-varicella zoster virus immunoglobulin have been proved to give significant results. Thus, it is of the utmost importance to provide information to parents and adequate management of this type of pregnancy (AU)

Australian Paediatric Surveillance Unit annual report, 2013.

This report provides an update on the surveillance conducted by the Australian Paediatric Surveillance Unit (APSU) during the period January to December 2013. The APSU facilitates national active surveillance of uncommon diseases of childhood including selected communicable diseases. This report includes data on the following conditions: acute flaccid paralysis (AFP), congenital cytomegalovirus (cCMV), congenital rubella, perinatal exposure to HIV and paediatric HIV infection, neonatal herpes simplex virus (HSV), congenital varicella, neonatal varicella, severe complications of varicella and juvenile onset recurrent respiratory papillomatosis (JoRRP). Surveillance of severe complications of influenza was undertaken during the influenza season (July to September 2013).

Anesthetic implications of two genetic translocation (6p21; 6p13) in a patient with history of congenital varicella syndrome: a case report.

The coexistence of multiple chromosomal abnormalities and Congenital Varicella Syndrome (CVS) in one patient is a rare event in which anesthetic implications should be considered. This case report describes a 9-year-old female with CVS and a karyotype analysis of 6p21; 16p13 genetic translocations. We conducted a detailed investigation of the consequences of such findings and the potential outcomes in anesthesia of this uncommon incident including thorough research on the characteristics present in each condition. We concluded that: (1) coexistence of two genetic translocations (6p21; 16p13) in one patient, and simultaneously with CVS is undoubtedly an extremely rare event; (2) difficult airway management, potential cardiac dysfunction, risk of pulmonary aspiration, fluid disturbances, and a hard to access peripheral vascularity are among the most important anesthetic implications as a consequence of having all these disorders; (3) ketamine was a safety and efficacious option for sedation during fiber optic bronchoscopy.

Varicella-zoster virus: Prevention through vaccination.

Widespread use of varicella vaccine in the United States has drastically changed the epidemiology of the disease. Although chickenpox is no longer a ubiquitous childhood infection, varicella-zoster virus continues to circulate in the community and nonimmune pregnant women remain at risk. Varicella can cause severe infection in pregnant women, often complicated by viral pneumonia. Maternal varicella occurring in the first half of pregnancy can cause the rare but devastating congenital varicella syndrome, whereas infection in the late stages of pregnancy may cause neonatal varicella. The best approach to avoiding the morbidity and mortality associated with chickenpox in pregnancy is to screen and vaccinate susceptible reproductive-age women.

Prenatal sonographic diagnosis of congenital varicella syndrome.

Congenital varicella syndrome is a rare disorder occurring in less than 1% of maternal varicella during early pregnancy but is associated with high fetal morbidity and mortality. This case report aimed to describe the sonographic features of congenital varicella syndrome following maternal varicella. Well-documented maternal chicken pox was made at 12 weeks of gestation and prenatal ultrasound was performed at 16 weeks. Striking sonographic features included hydropic changes and disseminated calcifications in multiple organs, especially liver and myocardium. Elective termination of pregnancy was done at 17 weeks. The presence of disseminated calcifications could suggest the diagnosis of congenital varicella syndrome.

Diagnóstico de infección congénita / Diagnosis of congenital infection

El diagnóstico de la infección congénita está basado en: a) serología materna; b) estudio microbiológico del líquido amniótico y sangre fetal, y c) serología en el neonato y detección del agente etiológico por cultivo o PCR. La infección congénita por citomegalovirus, virus herpes simple, virus varicela-zóster, Toxoplasma gondii y erythrovirus B19 suele ser el resultado de la infección primaria en la madre. Por lo tanto, la detección de anticuerpos IgG antes del embarazo permite descartar las infecciones por estos agentes. El diagnóstico serológico definitivo de infección aguda en la embarazada requiere la demostración de seroconversión. En tales casos, debe realizarse el estudio de infección congénita intrauterina en muestras de líquido amniótico y sangre fetal.Las infecciones por citomegalovirus, virus de la rubéola y T. gondii pueden diagnosticarse por detección de IgM en sangre fetal. Sin embargo, la PCR en líquido amniótico ha desplazado a las técnicas convencionales en el diagnóstico de estas infecciones. En el recién nacido, el diagnóstico puede confirmarse mediante detección de IgM específica.Erythrovirus B19 puede detectarse por PCR en líquido amniótico o sangre fetal.En la infección congénita por el virus varicela-zóster, la persistencia de IgG después del nacimiento permite establecer el diagnóstico.La detección directa del virus herpes simple en vesículas o muestras orofaríngeas es la técnica de elección para el diagnóstico de infección congénita por este agente(AU)

In general, congenital diagnosis is based on: a) maternal serologic assays; b) microbiologic study of amniotic fluid or fetal blood sampling; and c) serology in children and microorganism detection by polymerase chain reaction (PCR) or culture.Congenital infections due to cytomegalovirus, herpes simplex, varicella, B19 erythrovirus and toxoplasmosis are usually the result of primary infection in the mother. Therefore, when IgG antibodies are detected before pregnancy, these infections are ruled out. Definitive serologic diagnosis of acute infection in pregnant women requires the demonstration of seroconversion (i.e., from seronegative to seropositive). In these cases, amniotic fluid or fetal blood sampling should be performed to determine the presence of intrauterine congenital infection.Cytomegalovirus, rubella and toxoplasmosis can be diagnosed by detection of specific IgM antibodies in fetal blood. However, PCR in amniotic fluid has replaced conventional prenatal diagnostic techniques, including fetal blood sampling, in the diagnosis of these infections. In the newborn, these infections may be confirmed by measuring IgM specific antibodies.B19 erythrovirus can be detected by PCR in amniotic fluid or fetal blood. Congenital varicella-zoster infection may be diagnosed on the basis of persistence of IgG antibodies after birth. Definitive diagnosis of herpes simplex virus infection requires viral isolation. Swabs or scraping from clinical specimens can be inoculated into susceptible cell lines for isolation(AU)

[Varicella during pregnancy: consequences for the mother and the newborn]. / Varicelle pendant la grossesse: quelles conséquences pour la mère et l'enfant?

In our area, varicella is a frequent and essentially benign childhood disease. In contrast, the disease course is likely to be more severe or complicated in the adult, particularly so in the pregnant woman. There is a definite risk of congenital varicella syndrome when the chickenpox occurs during the first 20 weeks of pregnancy. This syndrome predominantly affects the skin, the subcutaneous tissue, muscles and bones, as well as the central nervous system, and can bring about major functional sequellae. In case of chickenpox occurring at the very end of pregnancy, transplacental transfer of the virus may result in a perinatal varicella disease. We propose a approach of each of these different situations.

Varicella-zoster virus (chickenpox) infection in pregnancy.

Congenital varicella syndrome, maternal varicella-zoster virus pneumonia and neonatal varicella infection are associated with serious fetomaternal morbidity and, not infrequently, mortality. Vaccination against varicella-zoster virus can prevent the disease, and outbreak control limits the exposure of pregnant women to the infectious agent. Maternal varicella-zoster immunoglobulin administration before rash development, with or without antiviral medication, can modify the progression of the disease.