Abstract
Background: Spherophakia is a rare condition and compared to its occurrence with familial and systemic disorders, isolated spherophakia is even more uncommon. This rare case of isolated spherophakia will raise the alertness of physicians when dealing with a high myopia patient with shallow anterior chamber and relatively normal fundus.
Case presentation: We report a case of a 17-year-old male who experienced painless decrease of vision in both eyes (OU) for 11 years, with progression of visual impairment and occasional ocular pain for one year. Examination revealed high myopia, increased intraocular pressures (IOP, 28 mmHg in the right eye (OD) and 33 mmHg in the left (OS)), shallow central anterior chambers, lenses of a spherical shape with superior subluxation, occludable angles without peripheral anterior synechiae, and healthy optic discs OU. A diagnosis of bilateral isolated spherophakia, lens subluxation, high myopia and secondary angle closure (AC) was made. Pars plana lensectomy with anterior vitrectomy and scleral suturing of an intraocular lens was performed. On postoperative follow-up at 19 days OD and 63 days OS, the visual acuity was 6/6 and the IOP was normal without any medication OU.
Conclusions: A presentation with high myopia, shallow anterior chamber with a normal retina should alert the clinician to the possibility of spherophakia, as compared to high myopia caused by elongated axial length. Surgery should be considered in cases of spherophakia with AC where the IOP cannot be controlled by noninvasive means.
The association of Fuchs's corneal endothelial dystrophy with axial hypermetropia, shallow anterior chamber, and angle closure glaucoma.
