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S. Sodhi, S. H. K. Reddy, A. R. Djalilian, S. H. K. Reddy; Morphological Lens Changes in Marfan Syndrome. Invest. Ophthalmol. Vis. Sci. 2009;50(13):4386.
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© ARVO (1962-2015); The Authors (2016-present)
Marfan syndrome is an autosomal dominant heritable disorder of fibrous connective tissue with marked pleiotropism and clinical variability of cardinal skeletal, cardiovascular, and ocular abnormalities. Considering the clinical variability of phenotypes, detection of ocular structural abnormalities can aid in the diagnosis of Marfan syndrome. While known ocular lesions occurring in patients with Marfan syndrome include ectopia lentis (50-80%), enophthalmos, megalocornea, keratoconus, iris hypoplasia, retinal lattice changes, and retinal tears, there is no literature describing the morphological changes of the lens in such patients. Here we present a case series of patients with known Marfan syndrome to specifically examine lens changes for aberrations in location, size, and shape.
Eight patients come from one geographic region known to have a large number of families affected with Marfan syndrome. All surgeries were performed by a single surgeon over a 5 year course in intracapsular fashion. Photographs were taken of the explanted lenses and they were grossly examined.
Patients ages range 31 -62 with a mean of 52. Pre-operative vision ranged from 20/70 to 20/800. Abnormalities in found include superior dislocation of lens, microphakia, and deranged shapes including kidney shape, spindled shape, or inferiorly flattening. These finding were consistent across all the explanted lenses, as seen in the photographs.
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