MANAGING ECTOPIA LENTIS

Lens subluxation occurs when there is partial disruption or distension of the zonular fibers, leaving the lens displaced from its normal position. When subluxation is associated with hereditary diseases with development defects, it is called Ectopia Lentis (EL).

The clinical presentation and respective treatment depend on several factors, such as: age of presentation, degree of subluxation, best corrected visual acuity (BCVA) and associated ocular defects.

The aim of this work is to describe the therapeutic strategies implemented in EL cases observed at the Hospital Professor Doutor Fernando Fonseca, the associated challenges and complications, as well as the visual results of the different approaches.

Retrospective analysis of 41 eyes of 24 patients with EL (32 cases associated with Marfan's Syndrome and 9 with Isolated Congenital Ectopia Lentis), observed between 2010 and 2020.

13 cases remained under conservative treatment, with a mean BCVA of 6/10 on the Snellen scale.

28 cases underwent lens extraction, with a mean age at surgery of 17.7 years. The mean preoperative BCVA was 2/10. In 17 eyes, the capsular bag (CB) was stabilized and centered, using a modified capsular tension ring (mCTR) with scleral fixation, followed by the implantation of a posterior chamber (PC) intraocular lens (IOL). In 7 cases, it was not possible to guarantee capsular support, opting for the implantation of an anterior chamber (AC) IOL with iris claw (n=3) or a PC-IOL with scleral fixation (n=4). In 4 cases, an intracapsular lens extraction with complete anterior vitrectomy was performed, with IOL not being implanted (n=1) or with AC-IOL implantation (n=3). Postoperative BCVA was 6/10+2 letters, with a mean gain of 4 lines+4 letters.

The complications observed were: partial extrusion of mCTR (n=2), extrusion of the scleral suture (n=1), subluxation of the IOL (n=4), opacification of the posterior capsule (n=5), phimosis of anterior capsule (n=2) and retinal detachment (n=3).

There is no statistically significant difference (p>0.05) between the choice of surgical strategy or the IOL implanted and visual gain.

There is no consensus on the best approach to EL. In cases where BCVA is ≥ 5/10 and no associated ocular pathology, correction of refractive error and periodic surveillance may be an option. When BCVA is not adequate or there are associated complications, surgical treatment should be recommended, with CB preservation and PC-IOL implantation being the first choice in our hospital.

EL commonly presents during childhood, is progressive and is associated with several anatomical and structural changes. These facts make this pathology a challenge for the most experienced surgeon, who may have to adapt the surgical technique according to the complications that may arise.