Bilateral Isolated Spherophakia in Two Young East European Siblings: A Case Report

A 19-year-old Caucasian woman was referred to the emergency room and thereafter to the department of ophthalmology complaining for bilateral decrease of visual acuity and severe pain. A complete ophthalmological evaluation was performed. Best-corrected visual acuity (BCVA) was LogMAR 0.3 in the right eye (RE) and LogMAR 0.5 in the left eye (LE). Intraocular pressure (IOP) was 28 and 38 mm Hg in the RE and LE, respectively. The patient showed a shallow anterior chamber and spherical equivalent refractive error −29.0 diopters (D) in the RE and −30.0 D in the LE. The diagnosis of bilateral angle closure glaucoma, secondary to highly myopic, forward dislocated lens was made, in the setting of spherophakia. The ultra-sound biomicroscopy images confirmed the diagnosis. Clear lens extraction was promptly performed with resolution of ocular hypertension and restoration of BCVA. In view of the frequent systemic association, family members also underwent ophthalmological evaluation. The 13-year-old sibling showed mild myopia and borderline IOP. He was administered topical β-blockers and observation. Genetic counseling did not reveal mutations usually associated with spherophakia or systemic conditions. This case report highlights the variable spectrum of clinical expression in spherophakia; therefore, ophthalmological treatment should be tailored according to clinical presentation. Systemic evaluation and genetic counseling are also recommended in the suspicion of spherophakia.

Aphakic Pupillary Block by an Intact Anterior Vitreous Membrane after Total Lens Extraction by Phacoemulsification

An 85-year-old Japanese woman with acute primary angle closure in her right eye underwent cataract extraction. Because of the weakness of the Zinn’s zonules, all of the lens tissue including the lens capsule was removed by phacoemulsification. Because of the absence of vitreous prolapse into the anterior chamber, vitrectomy was not performed. Nine days postoperatively, acute angle closure due to pupillary block by an anterior vitreous membrane developed. To resolve the pupillary block, anterior vitrectomy was performed on the same day. Postoperatively, her symptoms resolved, the anterior chamber deepened, and the intraocular pressure normalized. Although rare, acute angle closure due to pupillary block by an anterior vitreous membrane can occur after total lens extraction with phacoemulsification. If no vitreous prolapse occurs with total lens extraction, an intentional hyaloidotomy using an anterior vitreous cutter or iridectomy should be considered to avoid secondary angle closure.

Adapted Bag-in-the-Lens Implantation Technique in Children with Congenital Ectopia Lentis

Background Subluxation of the crystalline lens in childhood confronts the surgeon with a dilemma: to operate or to wait and see. Surgery is usually not performed when the subluxation is still limited. However, postponing the surgery increases the surgical difficulty as the capsular bag becomes more difficult to use as a means of support for the intraocular lens (IOL). A large number of children already present a pronounced subluxation at first presentation. In this paper, we describe a technique to optimise centration and fixation of the bag-in-the-lens (BIL) IOL in children younger than 7 years of age with congenital ectopia lentis. Methods Between October 2019 and December 2020, we performed lens extraction using a combination of bean-shaped segments to support the BIL IOL and a 6 – 0 polypropylene loop fixated at the sclera, following the Yamane technique, for the purpose of centration. We used this technique for seven eyes of four patients. The patients were between 2 and 6 years old; 3 boys and 1 girl. A definite diagnosis of Marfan syndrome was made for two children; for the other two, there was no proven underlying pathology. The luxation was upwards in all cases. The degree of luxation was severe in all eyes. The preoperative refraction values showed high astigmatism values for all eyes, ranging from 6.5 to 11.25 dioptres. Three out of the four patients were myopic, ranging from − 1.5 to − 9 dioptres. Results The surgery could be performed without major complications in all eyes. Good centration was obtained, which remained stable in the postoperative period. Refraction improved with greatly diminished degrees of astigmatism (ranging from 0.25 to 3 dioptres) and myopia (spheres ranging from − 2 to + 1.75 dioptres). Conclusion Our novel technique incorporated the BIL technique with the addition of bean-shaped segments and a polypropylene 6/0 suture fixated at the sclera. In this way, we were able to obtain good centration and stability of the implanted IOL, as well as a good refractive outcome in all cases.

Visual outcomes and patient satisfaction 1 and 12 months after combined implantation of extended depth of focus and trifocal intraocular lenses

Purpose To assess the 1-month and 12-month postoperative visual performance and subjective outcomes following combined implantation of an extended depth of focus (EDOF) intraocular lens (IOL) and a trifocal IOL. Methods The study enrolled consecutive patients undergoing refractive lens extraction or cataract surgery with combined implantation of an EDOF IOL (dominant eye) and trifocal IOL. Uncorrected (UDVA) and best-corrected (CDVA) distance visual acuities, uncorrected intermediate (UIVA) and near (UNVA) visual acuities, and subjective questionnaires were evaluated 1 month and 12 months postoperatively. Results The study enrolled 58 consecutive patients. Binocular UDVA, UIVA and UNVA were − 0.08 ± 0.07 logMAR, 0.15 ± 0.14 logMAR and 0.17 ± 0.11 logMAR at 1 month, compared to − 0.09 ± 0.06 logMAR (P = .323), 0.11 ± 0.10 logMAR (P = .030) and 0.13 ± 0.10 logMAR (P = 0.008) at 12 months. Satisfaction was high with 93.1% of patients fulfilled or more than fulfilled postoperatively, and 84.5% and 86.3% reported spectacle independence for near at the respective postoperative assessments. The mean daytime and nighttime quality of vision (QoV) scores were 9.12 ± 0.94 and 7.88 ± 1.74 at 1 month, compared to 9.24 ± 0.78 (P = .183) and 8.26 ± 1.38 (P = .043) at 12 months. Conclusions This IOL combination provides good unaided visual acuity at 1 and 12 months postoperatively, with high functional vision and postoperative satisfaction reported at 1 and 12 months. However, a significant improvement in overall nighttime QoV at the 12 months assessment was found.