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Ocular Manifestations of the Congenital Varicella Syndrome
Scott R. Lambert, MD;
David Taylor, FRCS, FRCP;
Anthony Kriss, PhD;
Helen Holzel, MScMD, MRCPath;
Shelley Heard, PhD, MRCPath
Arch Ophthalmol. 1989;107(1):52-56.
Abstract
• 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.
Author Affiliations
From the Departments of Ophthalmology (Drs Lambert, Taylor, and Kriss) and Microbiology (Drs Holzel and Heard), The Hospital for Sick Children, London. Dr Lambert is now with Emory University Eye Center, Atlanta.
Footnotes
Accepted for publication May 27, 1988.
Reprint requests to Department of Ophthalmology, The Hospital for Sick Children, Great Ormond St, London WC1N3JH, England (Dr Taylor).
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