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Additional information that contributes to our Clinicopathologic Report¹ has come to our attention. We described zonule-associated staining of matrix metalloproteinases (MMPs) in Marfan syndrome lens subluxation. We hypothesized that the product of the defective fibrillin-1 gene in Marfan syndrome is more prone to degradation by MMPs than normal fibrillin and that an imbalance of MMPs and their inhibitors could result in the progressive destruction of lens zonules and subsequent lens subluxation.

Clinically, in patients with Marfan syndrome, lens subluxation tends to occur in the superotemporal direction.² Additionally, lens subluxation tends to occur in older Marfan patients.³ The reason for this pattern of dislocation and the age-related incidence of this problem remains unknown.

The anterior segment peripheral light focusing effect⁴ predicts that UV-B light is preferentially focused toward the inferonasal quadrant of the crystalline lens (Figure 1, A). Thus, lens zonules may be differentially exposed to cumulative UV-B exposure.⁵ . . . [Full Text of this Article]

Corresponding author: Minas T. Coroneo, FRACO, FRACS, MD, Department of Ophthalmology, Prince of Wales Hospital, University of New South Wales, Randwick, Sydney, Australia (e-mail: m.coroneo@unsw.edu.au).