Surgical Outcomes of Lens Removal with or Without Intraocular Lens Implantation in Marfan Syndrome: A Retrospective Cohort Study.

PURPOSE: To evaluate the outcomes of lensectomy with and without intraocular lens (IOL) implantation in patients with Marfan syndrome and ectopia lentis.

METHODS

This retrospective review included 55 patients (85 eyes) diagnosed with Marfan syndrome who underwent lens surgery at King Khaled Eye Specialist Hospital between 2014 and 2023. Preoperative lens status; surgical technique; use of capsular support devices and IOLs; and postoperative clinical, biometric, and refractive parameters were analyzed. The primary outcome was final best-corrected visual acuity. Secondary outcomes included refractive correction (spherical equivalent), axial length, intraocular pressure, IOL position and stability, and incidence of postoperative complications.

RESULTS

All eyes had subluxated crystalline lenses [predominantly inferotemporal lenses (52.9%)]. Surgical procedures included lensectomy with anterior vitrectomy (49.4%), lensectomy with IOL implantation (38.8%), pars plana vitrectomy with lensectomy (7.1%), and phacoemulsification with IOL (4.7%). Capsular support devices were used in 12 eyes. Secondary IOL implantation was performed in 12 eyes, with 91.7% scleral-fixated IOLs. At the final follow-up, 41.2% of the eyes were aphakic, 32.9% had scleral-fixated IOLs, 15.3% had posterior chamber IOLs, and 8.2% had anterior chamber IOLs. There was a significant association between phakic status and refractive correction (P<0.001). The final mean visual acuity improved significantly from 1.1±0.7 logMAR (20/250) at presentation to 0.3±0.3 logMAR (20/40) at the last visit (P<0.001). The IOL position was stable in 47.1% of the eyes and was significantly associated with IOL type (P<0.001).

CONCLUSION

Lensectomy, with or without IOL implantation, is an effective and versatile approach for managing ectopia lentis in Marfan syndrome, with favorable long-term visual and anatomical outcomes. These findings emphasize the need for individualized planning based on subluxation severity and capsular support. Improved IOL stability and vision support the use of tailored techniques. Future studies should assess long-term safety and quality of life to guide standardized care.