The congenital varicella syndrome

Borzyskowski, Malgorzata; Harris, R.; Jones, R. Watson

doi:10.1007/bf00443268

Cited by 41 publications

(5 citation statements)

References 16 publications

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“…Childhood Horner’s syndrome has been described in congenital varicella,5 6 birth trauma,7 tumours of the neck and mediastinum such as neuroblastoma,8-10ganglioneuroma11 12 and neurilemmoma,13 and vascular lesions of the internal carotid or subclavian artery 14. Although many reports recommend extensive investigation of these children, they do not clearly distinguish congenital isolated Horner’s syndrome from that acquired later in childhood accompanied by other signs at presentation.…”

mentioning

confidence: 99%

Does Horner's syndrome in infancy require investigation?

George

Gonzalez²,

Hoyt³

1998

British Journal of Ophthalmology

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Aims-To evaluate whether isolated Horner's syndrome presenting in the first year of life warrants investigation. Method-Retrospective review of 23 children presenting with Horner's syndrome in the first year of life. Results-In 16 patients (70%) no cause was identified. Birth trauma was the most common identifiable cause (four patients). Twenty one children (91%) had urinary vanillylmandelic acid (VMA) measured and 13 patients (57%) underwent either computed tomography or magnetic resonance imaging of the chest and neck. These investigations revealed previously undisclosed pathology in only two-one ganglioneuroma of the left pulmonary apex and one cervical neuroblastoma. A further patient was known to have abdominal neuroblastoma before presenting with Horner's syndrome. There were no cases of Horner's syndrome occurring after cardiothoracic surgery. Long term follow up of the patients (mean 9.3 years) has not revealed further pathology. Conclusions-Routine diagnostic imaging of isolated Horner's syndrome in infancy is unnecessary. Infants should be examined for cervical or abdominal masses and involvement of other cranial nerves. If the Horner's syndrome is truly isolated then urinary VMA levels and follow up in conjunction with a paediatrician should detect any cases associated with neuroblastoma. Further investigation is warranted if the Horner's syndrome is acquired or associated with other signs such as increasing heterochromia, a cervical mass, or cranial nerve palsies. (Br J Ophthalmol 1998;82:51-54) Horner's syndrome occurs as a result of a lesion anywhere along the oculosympathetic pathway from the hypothalamus to the orbit.

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confidence: 99%

Does Horner's syndrome in infancy require investigation?

George

Gonzalez²,

Hoyt³

1998

British Journal of Ophthalmology

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“…No change in the diagnosis of CVS was made. Clinical characteristics of the reported 15 patients, including our patient, are listed in Table 1 [2,9,10,11,12,13,14,15,16,17,18,19,20]. The most frequent anomaly is neurogenic bladder.…”

Section: Discussionmentioning

confidence: 99%

Genitourinary anomaly in congenital varicella syndrome: case report and review

et al. 2004

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We describe a 1-year-old boy with congenital varicella syndrome who had vesicoureteral reflux (VUR) and neurogenic bladder. His mother had varicella during the 3rd month of pregnancy. At birth the patient presented with right microphthalmia, right microcornea, and persistent hyperplastic primary vitreous of the right eye. He had chronic constipation from 3 months of age. He had urinary tract infection at 1 year of age. Urological investigation revealed left grade V VUR and neurogenic bladder. His varicella zoster virus IgG titer measured by ELISA was 39.4 antibody index (normal <0.1). He had repeated episodes of urinary tract infection despite antibiotic prophylaxis and clean intermittent catheterization, and underwent a uretero-vesiconeostomy at 2 years of age. Maternal infection during early pregnancy and the serological evidence of varicella zoster IgG antibodies without a history of varicella after birth led to the diagnosis of congenital varicella syndrome. Urogenital anomalies have previously been described in 14 cases of congenital varicella syndrome. Most of these patients had neurogenic bladder, the pathophysiology of which could be explained by the known neurotropic nature of the virus.

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“…Genetic factors in the fetus may play a role in the fetal immune response, but there has been no study to date. Interestingly, in a case of monochorionic twins, one had typical FVS while the other twin was healthy at 3 years follow‐up. In cohorts of FVS, there is a predominance of females, 66% to 85% .…”

Section: Fetal Varicella Syndromementioning

confidence: 99%

Fetal varicella – diagnosis, management, and outcome

Mandelbrot

2012

Prenatal Diagnosis

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Fetal varicella syndrome (FVS) is due to transplacental infection by the Varicella zoster virus following maternal infection. The risks for the fetus and neonate depend on the timing. When varicella occurs around delivery, it often leads to disseminated neonatal varicella. When varicella occurs during pregnancy, transmission can occur, but is usually asymptomatic; some infants develop zoster postnatally and a few have FVS. Before 20 weeks' gestation, FVS can occur, with an incidence of about 1%. The lesions can affect the skin, limbs, central and autonomous nervous systems, eyes, cause calcifications, and growth retardation; mortality is high. Lesions typically follow one or several nerve territories, suggesting that damage results from in utero zoster following primary fetal infection. There has been little study of prenatal diagnosis of FVS. Serial ultrasound examination can detect various anomalies, magnetic resonance imaging can be of use to investigate for microphthamia and cerebral lesions, and amniocentesis can diagnose viral transmission. Prevention strategies include vaccination and post-exposure prophylaxis with immune globulin and/or antivirals. Perspectives for treating infected fetuses in utero require further research.

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The congenital varicella syndrome

Cited by 41 publications

References 16 publications

Does Horner's syndrome in infancy require investigation?

Does Horner's syndrome in infancy require investigation?

Genitourinary anomaly in congenital varicella syndrome: case report and review

Fetal varicella – diagnosis, management, and outcome

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