Spherophakia in a Newfoundland family: 8 years' experience

In a family with eight siblings, five siblings have had spherophakia. One patient has died, but the other four have been followed up for 8 years. Two have undergone peripheral iridectomy because of steadily rising intraocular pressure. The operations were only partially successful in controlling the pressure. Whether associated with the Weill-Marchesani syndrome or not, spherophakia is difficult to manage. The lenticular myopia must be corrected (sometimes an additional reading correction is needed), and the intraocular pressure and visual field must be carefully monitored. The ability of the retinal circulation to withstand a high intraocular pressure for long periods may not be matched by a corresponding resilience of the drainage mechanism. Therefore, early peripheral iridectomy is probably advisable.