Ectopia lentis a major ocular manifestation of Marfan syndrome

Marfan syndrome is an autosomal dominant genetic disorder caused by the misfolding of the protein fibrillin-1. It is also a connective tissue disorder with varied expressions in the body with systemic and ocular involvement.The major ocular finding of Marfan syndrome (MFS) is ectopia lentis, and approximately 60% of patients have lens dislocation, which is usually superior and temporal; this may be present at birth or develop during childhood or adolescence. At least two minor criteria must be present for consideration as ocular involvement: an abnormally flat cornea, increased axial length, (Read more...)

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