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.

Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report

“…Spherophakia is a congenital eye disorder, usually bilateral, that presents with increased anteroposterior diameter (a more spherical lens), varying degrees of lenticular myopia, and zonular weakness [6]. Spherophakia can occur isolated, affect multiple family members, or be associated with homocysteinemia, hyperlysinemia, Weil-Marchesani, Marfan, and Alport syndromes [7, 8].…”

“…Spherophakia can occur isolated, affect multiple family members, or be associated with homocysteinemia, hyperlysinemia, Weil-Marchesani, Marfan, and Alport syndromes [7, 8]. Among affected cases, hypoplastic lens zonules fibers are conducive for lens subluxation or dislocation from the fossa leading to pupillary block glaucoma [6, 9].…”

Sutureless Scleral-Fixated Intraocular Lens Implantation for Refractive Rehabilitation in Eyes With Spherophakia

“…3 Patients with spherophakia may seek treatment because of high lenticular myopia, subluxation of the lens and defective vision, and secondary glaucoma, and lensectomy has been shown to be therapeutic in most cases. 4 The most common time of presentation of spherophakia in patients is in early adulthood or adolescence. However, in patients with systemic associations, the disease may present earlier in life.…”

Subluxated spherophakic lens: Zonules still not relinquished

“…Microspherophakic lens are small with weak zonules, leading to clinical manifestations of high myopia, progressive subluxation and/or dislocation, and glaucoma either pupillary block or angle closure. [1]…”