ABSTRACT

Microspherophakia is a developmental disorder of the lens characterized by a small and spherical lens. Defective development of the lens fibers during embryogenesis was thought to be responsible for the abnormal size and shape of the lens. Weill-Marchesani syndrome (WMS) is the most common syndrome accompanying microspherophakia. The spherical shape of the lens increases the refractive power of the lens and leads to high myopia. Furthermore, due to zonular weakness, the small and spherical lens can easily move forward and lead to a pupil block. In this case report, we discussed a case of WMS with microspherophakia in which the yttrium-aluminium-garnet (YAG) laser iridotomy had been performed on multiple areas to prevent pupillary block, but glaucoma progressed despite multiple laser iridotomy treatments. We stressed that if YAG laser iridotomy is implemented as first-line treatment in microspherophakic patients with WMS, the patients should be closely monitored. We also emphasized that if glaucoma progression continues despite the effective YAG laser iridotomy, the ophthalmologist should not insist on YAG laser treatment, and surgical lens removal option should be considered.

Keywords: Microspherophakia, pupillary plock glaucoma, Weill-Marchesani syndrome, yttrium-aluminium-garnet laser iridotomy