OCULAR MANIFESTATIONS
The FBN 1 gene produces microfibrils that support the lens and keep it in position, the microfibrils are also found in the iris, Schlemm canal and cornea. The defect in these microfibrils is associated with ectopia lentis (lens subluxation), the development of miosis, wider corneal diameter, and pupillary hypoplasia 9-11
Myopia is the commonest ocular manifestation of the disease, being associated with elongation of the globe with 60% of these having concomitant lens dislocation 12, 13. The incidence of ectopia lentis in Marfan syndrome varies from 30% to 72% in different studies it may however be first seen in the second decade of life14-16.
Less common ocular manifestations are early cataracts, retinal detachment, and glaucoma.  Ocular abnormalities such as myopia, astigmatism, anisometropia, ectopia lentis, and retinal pathologies may result in amblyopia. The earlier the onset and the longer it goes untreated, the harder amblyopia is to correct 17. Therefore, patients with MFS should be screened annually and examined for refractive errors and other ocular pathologies. Eyeglasses are the first step to correct blurred vision caused by subluxated lenses18. Removal of a dislocated lens and the possible insertion of an artificial one may be considered but not usually required 14.
The indications for surgery in lens dislocation are inability to achieve good corrected visual acuity, risk of amblyopia in children, posterior dislocation of the lens into the vitreous cavity, anterior dislocation of the lens with or without secondary glaucoma, impending to complete lens dislocation, lens induced glaucoma or uveitis, and cataract19.
Glaucoma may develop at a later age and may be as a result of phacolytic glaucoma from dislocation of a matured lens 20, 21.