Kamath, Gurudutt M and Nayak , Madhurima A and Dudeja, Lakshey (2014) Anteriorly Dislocated Lens in Weill Marchesani Syndrome. International Journal of Bioassays, 3 (06). pp. 3048-3050. ISSN 2278-778X

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Abstract

Ectopia lentis has many systemic associations, Weill Marchesani syndrome being one of them. It is a rare connective tissue disorder, characterised by a small, spherical lens (Microspherophakia); short fingers (Brachydactyly) and short stature (Brachymorphia). We present a case of the same, in a 55 year old woman who presented with painful diminution of vision and her son noticed cataract in her left eye. She had undergone cataract surgery for the right eye previously. On examination, the best corrected visual acuity was 6/6 in right eye, and perception of hand movements in left eye. Anterior segment of left eye showed mild central corneal edema, anterior chamber reaction and anteriorly dislocated spherical cataractous lens. She had short stature and short stubby fingers. A clinical diagnosis of Weill Marchesani syndrome was made. She underwent lens extraction with ACIOL implantation. Post operatively, corneal edema resolved and visual acuity was 6/9. Thus, prompt lens extraction is needed to prevent corneal endothelial damage. We report a case of anterior dislocated cataractous lens in an elderly lady.

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