Ectopia lentis and other systemic features of Marfanoid habitus

Marfan syndrome is one of the most common inherited connective tissue disorders, affecting multiple organ systems, including the eye. A mutation in the gene fibrillin-1 contributes to the pathogenesis of the classical manifestations of this systemic disorder. Among all ocular features, ectopia lentis (EL) is the most common finding leading to decreased vision. EL has an association with many other syndromic disorders affecting connective tissues or can be present as an isolated one. Management of subluxated lens with appropriate optical correction such as spectacles, aphakic glasses, scleral-fixated intraocular lens (IOL), or iris-fixated IOL, which is individualized according to the severity. Prompt recognition of multisystem dysfunctions holds the key to enhancing the lifespan of the patient and quality of life as well.