Ectopia Lentis et Pupillae is a rare, autosomal recessive condition primarily linked to mutations in the ADAMTSL4 gene located on chromosome 1. These mutations disrupt the development and stability of the zonular fibers which are essential for suspending the lens. Unlike Marfan syndrome, this is an isolated ocular anomaly where the hallmark is the eccentric displacement of the lens and the pupil in opposite directions. Surgical management of the associated zonulopathy requires advanced stabilization techniques such as capsular and iris hooks which are first deployed to secure the bag and improve visualization. After lens removal, a Cionni Capsular Tension Ring is placed within the capsular bag. To ensure permanent centration, 6-0 Prolene sutures are fixated to the sclera using the flange technique, where cauterized suture bulbs are tucked into scleral tunnels. Finally, a pupilloplasty is performed to centralize the aperture. By suturing the iris tissue, the surgeon aligns the pupil with the stabilized intraocular lens, restoring the visual axis.
