We appreciate and thank Dr. Kosker for his interest in our article and for sharing his clinical observations. Kosker’s report of a case of xeroderma pigmentosum with prolonged corneal edema after an uneventful phacoemulsification in 1 eye and lower endothelial cell density in the opposite eye is suggestive of lower endothelial cell reserve, presumably due to ultraviolet radiation–induced damage in this condition. As suggested by Kosker, we also advocate specular microscopy as a routine investigation in patients with xeroderma pigmentosum to monitor the health of the amitotic corneal endothelium. In our clinical practice, we have been performing serial specular microscopy in all patients with xeroderma pigmentosum with clear corneas. We have observed that several of these patients have lower endothelial cell counts compared to the age-matched controls (unpublished data).

Kosher and associates report presenile mature cataract in patients with xeroderma; it is probably caused by improper working of the DNA repair mechanism of the lens epithelial cells, and the report is noteworthy. The normal cornea readily transmits ultraviolet radiation longer than 300 nm, which can reach the anterior segment and can cause iris and lens changes. However, none of the cases included in our article had concomitant lenticular changes; hence, we did not resort to triple procedures.

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Jan 8, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Reply

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