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Last Month’s Blink

Bilateral Ectopia Lentis in Suspected Marfan Syndrome

Written by Nitin K. Menia, MS, Reema Bansal, MS, and Sandeep Bansal, MS. Photos by Arun Kapil. All are at Advanced Eye Centre, Post Graduate Institute Of Medical Education and Research, Chandigarh, India.

A 13-year-old girl presented for visual assessment. Her BCVA was 20/30 in both eyes. IOP was 16 mm Hg and 14 mm Hg in the right and left eyes, respectively. The anterior segment examination showed superonasal sublux­ation of the crystalline lens with visible stretched zonules in both eyes (Figs. 1 and 2). Fundus exam­ination was unremarkable.

Systemic evaluation by the pediatrician re­vealed features suggestive of Marfan syndrome, including a small forehead, low-set ears, long tri­angular face with malar hypoplasia, microstomia, peaked nose, and high-arched palate with disor­ganized teeth. She had mild mitral valve and tri­cuspid valve regurgitation and bilateral conductive hearing loss. Homocystinuria and Weill-Marches­ani must also be considered in the differential diagnosis of ectopia lentis in a young person.

Because the patient had VA of 20/30, with­out anisometropia, significant astigmatism, or complications related to subluxated lenses (such as cataract, glaucoma, uveitis, or retinal detach­ment), her physicians determined that she did not require immediate treatment. She will receive regular ocular and pediatric follow-up to monitor for progression.

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