Keratoplasty in xeroderma pigmentosum.

نویسنده

P SIVASUBRAMANIAM

چکیده

KERATITIS is the chief ocular complication of xeroderma pigmentosum. The severe photophobia, a marked feature of this disease, and the extensive corneal involvement, mainly in the interpalpebral zone, make sufferers from this malady virtually blind, at any rate by day. In a report of seven cases of this disease with ocular complications (Sivasubramaniam and Hoole, 1952), keratoplasty was suggested as a means of treating the corneal condition, though at that time no case had been so treated. As there appears to be hardly any literature on this aspect of xeroderma pigmentosum, the following account of three patients treated by keratoplasty in the Government General Hospital, Jaffna, Ceylon, may be of interest. As far as keratoplasty is concerned the important lesions are a diffuse keratitis extending down to the anterior two-thirds of the substantia propria of the cornea, and telangiectases visible to the naked eye at and within the limbus. The depth of the keratitis was only discernible on the operating table as examination with the biomicroscope was precluded by the severe photophobia. Symblepharon may be encountered though this was not a problem in the cases described below.

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