Abstract
BackgroundThe 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.MethodsWe 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.ResultsThe 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.ConclusionScleral 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.
Highlights
The dislocation of the crystalline lens is a common finding in patients with Marfan syndrome (MFS)
The primary aim of this study was to assess whether scleral intraocular lens (IOL)-fixation in MFS
Our results show that both groups are comparable in regard to the refractive and visual outcomes but differ in their postoperative complications
Summary
The dislocation of the crystalline lens is a common finding in patients with Marfan syndrome (MFS). Marfan syndrome is a rare genetic disorder of the connective tissue, with an incidence of one to two per 10.000 individuals [1, 2]. It was first described by the French pediatrician Antoine-Bernard Marfan in 1896 [3]. MFS affects the cardiovascular, skeletal, and ocular system with high clinical variability [6] While cardiovascular complications, such as aortic aneurysm and mitral valve prolapse, are life threatening, abnormalities in the skeletal system, such as long and thin extremities with spidery fingers and toes, account for the typical clinical picture of MFS [2, 7].
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