マルファン症候群の眼合併症に対する外科的アプローチ

Marfan syndrome is a connective tissue disorder with several vision-threatening ocular manifestations. This study synthesizes recent advances in the surgical approach to ocular complication of Marfan syndrome including ectopia lentis, early cataract, glaucoma, and retinal detachment.

Recent literature highlights advances in capsular support devices and alternative fixation methods for ectopia lentis, including long-term outcomes with modified capsular tension rings, suture-less scleral fixation, and iris-claw intraocular lenses. Pediatric cohorts underscore elevated risks of retinal detachment following lens removal, particularly when capsular remnants persist, emphasizing the importance of complete removal and vigilant follow-up. In highly myopic Marfan eyes, modern intraocular lens power calculation formulas demonstrate improved refractive predictability, though pediatric patients remain prone to progressive myopic shift. For glaucoma, tailored modifications to trabeculectomy and tube shunt techniques address the challenges of thin sclera and ocular surface fragility. Contemporary retinal detachment series reveal high lifetime risk, with surgical success often requiring multiple procedures, and outcomes closely tied to macular status and presence of proliferative vitreoretinopathy.

Advances across anterior and posterior segment surgery have improved visual outcomes for Marfan patients, but long-term risks remain substantial. Individualized surgical planning, early detection of complications, and long-term surveillance are essential to optimize outcomes in this high-risk population.