Bilateral phacomorphic angle-closure glaucoma in a highly myopic patient secondary to isolated spherophakia

Angle closure most commonly occurs in older hyperopic patients as a result of primary relative pupil block. Less frequently, angle closure occurs in highly myopic patients with conditions other than primary relative pupil block. This report presents the diagnosis, pathophysiologic mechanism, and management of a patient with both high myopia and bilateral advanced phacomorphic angle-closure glaucoma caused by isolated spherophakia.

A 40-year-old asymptomatic man with very high myopic astigmatism presented with chronic angle closure and an intraocular pressure of 42 mmHg in both eyes. Additionally there was a nonmyopic fundus and 24-mm axial length, with a clear crystalline lens protruding through the pupillary plane in each eye, confirmed by B-scan ultrasonography. Gonioscopy and A-scan and B-scan ultrasonography identified the pathogenesis of intraocular pressure elevation, angle closure, and high myopia to be lenticular in origin. Initial medical therapy and subsequent laser iridotomy relieved the pupil block angle closure and successfully lowered intraocular pressure.

Angle closure can occur in highly myopic eyes. Careful gonioscopy and ultrasonography can lead to the correct diagnosis and tailored management for these eyes. Phacomorphic angle-closure glaucoma from spherophakia is associated with Weill-Marchesani syndrome as well as a few other uncommon syndromes. Isolated pseudophakia is a rarely reported cause of phacomorphic angle closure.