Marfan syndrome is a connective tissue disorder originating from a mutation in the fibrillin-1 gene. Ectopia lentis is a cardinal diagnostic sign of Marfan syndrome as previously described in the… Click to show full abstract
Marfan syndrome is a connective tissue disorder originating from a mutation in the fibrillin-1 gene. Ectopia lentis is a cardinal diagnostic sign of Marfan syndrome as previously described in the Ghent 1 criteria, and about 60 per cent of patients have lens dislocation. The current management for severe ectopia lentis is surgical correction. However, this may be technically challenging due to the unique inherent ocular anatomy associated with Marfan syndrome. These patients tend to have longer axial length, thicker lens, and flatter cornea. The most difficult surgical challenge originates from the weaker zonule fibres. Various operative approaches have been suggested, including the use of anterior chamber intraocular lenses. However, some patients with Marfan syndrome still suffer from visual problems after anterior chamber intraocular lens implantation. In this paper, we present an interesting case of Marfan syndrome who suffered from monocular diplopia due to a combined effect from a subluxated crystalline lens plus a decentred anterior chamber intraocular lens, which was resolved after an Artisan iris-claw intraocular lens substitution.
               
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