Ocular manifestations of the congenital varicella syndrome
S. R. Lambert, D. Taylor, A. Kriss, H. Holzel and S. Heard
Department of Ophthalmology, Hospital for Sick Children, London, England.
Three children developed the congenital varicella syndrome following a
maternal varicella infection during the second trimester of pregnancy.
Systemic findings included a bulbar palsy, mild hemiparesis, cicatricial
skin lesions, developmental delay, and learning difficulties. Ocular
findings included chorioretinitis, atrophy and hypoplasia of the optic
discs, a congenital cataract, and Horner's syndrome. Electroretinograms and
pattern-reversal visual-evoked potentials recorded from the four eyes with
chorioretinitis were attenuated and degraded. Two of the children had been
misdiagnosed as having congenital toxoplasmosis. Chorioretinal scars
probably occur more frequently with the congenital varicella syndrome than
has been previously recognized and should be included in the differential
diagnosis of congenital chorioretinal scars. Hypoplasia of the optic disc
may develop after an intrauterine varicella infection during the 16th week
of gestation. Children with the congenital varicella syndrome should be
examined by an ophthalmologist to exclude ocular abnormalities. These
abnormalities are often subtle and difficult to diagnose correctly.
