Long-term follow-up after scleral lens fixation in patients with Marfan syndrome

Jan Luebke, Thomas Reinhard, Hansjuergen Agostini, Daniel Boehringer, Philipp Eberwein

The dislocation of the crystalline lens is a common finding in patients with Marfan syndrome (MFS). Scleral intraocular lens (IOL) fixation is an accepted treatment method of this complication. To now, no long-term data on scleral IOL fixation in MFS exist. We present a retrospective study of 27 eyes of 17 MFS patients that underwent scleral lens fixation at our clinic between 1999 and 2012. These patients are compared to an age- and surgeon-matched group of 31 eyes of 27 patients who underwent the same procedure for reasons other than MFS. The median age in the MFS group was 35.4 years versus 35.6 years in the non-MFS group. The median follow-up was 4 years for MFS and 3 years for non-MFS. In the MFS group, significantly more IOL-dislocations occurred than compared to the non-MFS group (30% vs. 6.5%, p = 0.02). Retinal detachment occurred in four MFS-eyes compared to three eyes in the non-MFS group. Biometry prediction error was 1.11 diopters (D) for MFS and 1.33 D for non-MFS (p = 0.11). Median BCVA (best-corrected visual acuity, logMAR) was 0.1 in the MFS group versus 0.3 in non-MFS patients. Scleral lens fixation in MFS patients achieves satisfying visual and refractive outcomes. Our data shows a significantly higher rate of IOL dislocations in patients with MFS. We therefore recommend addressing this complication preoperatively.