Clinical spectrum and surgical outcomes in spherophakia: a prospective interventional study

Abstract

PurposeTo study the varied clinical presentations of patients with spherophakia, their management using surgical methods, and the clinical outcomes.Patients and methodsA prospective interventional study of 13 patients of spherophakia who presented to us from January 2014 and were followed up over the course of their treatment, and the data were documented for analysis.ResultsIn all, 26 eyes of 13 patients were reviewed and the median age of presentation was 12±12.05 years. All patients had a bilateral presentation with 22 eyes having lenticular myopia with a mean refractive error of -11.5±12.945 DS. Ten eyes presented with glaucoma of which six had raised intraocular pressure (IOP) >21 mm Hg. A total of 23 eyes underwent lens extraction for dislocation/subluxation. Lens extraction helped lower overall IOP. Refractive rehabilitation was done with ACIOL, posterior chamber intraocular lens (PCIOL) with capsular tension ring, and scleral-fixated intraocular lens (SFIOL) in respective cases with ACIOLs being the most commonly used option.ConclusionsSpherophakia is a rare condition, which exhibits a varying degree of lenticular myopia, glaucoma, and subluxation of the crystalline lens. Lensectomy with proper rehabilitation using ACIOL, PCIOL, or SFIOL is a method of managing subluxation and unacceptable myopia. Lensectomy may also be a viable option of controlling glaucoma alongside medications and glaucoma surgery for the management of glaucoma in such cases.

Figure 1

Endocapsular lens aspiration with insertion of ACIOL. (a) MVR entry is made in the cornea and extended to make a nick in the anterior capsule of the lens. (b) Two nicks are made in the anterior capsule with MVR blade. (c) Adrenaline is injected through one of the nicks for hydro dissection. (d) Irrigation cannula through opening and bag stabilized. (e) 25G vitrectomy cutter is inserted through one of the nicks and lens aspiration done in I–A mode. (f) Capsular bag is eaten and limited anterior vitrectomy is done with the cutter in cut I–A mode. (g) Pilocarpine followed by air is injected intracamerally and the iris is stroked with a Sinkey hook to constrict the pupil. (h) One of the corneal incisions is enlarged for the insertion of ACIOL and a PI is done. A full colour version of this figure is available at the Eye journal online.

Figure 2

(a) Anteriorly dislocated spherophakic lens. (b) UBM showing lens in anterior chamber touching the cornea. (c) ACIOL in spherophakic patient. (d) SFIOL in spherophakic patient. A full colour version of this figure is available at the Eye journal online.