Ectopia Lentis in Marfan Syndrome

A 27-year-old woman became extremely concerned when she was unable to pass the vision test required to obtain a driver's license. The patient had Marfan syndrome and “dislocated lenses.” She was otherwise healthy and had no cardiac abnormalities.

Developmental ectopia lentis (subluxation of the lens) occurs in 50% to 80% of persons with the autosomal dominant hereditary disease Marfan syndrome. The lens dislocation is usually supratemporal (Figures) and is almost always bilateral and fairly symmetrical. Other ophthalmic abnormalities that affect patients with Marfan syndrome include high myopia, retinal detachments, amblyopia, strabismus, and glaucoma.

Dr Leonid Skorin, Jr, of Dixon, Ill, cautions patients with ectopia lentis to avoid contact sports. Physical contact heightens the risk that the subluxated lens will migrate into the anterior chamber or vitreous and increases the chance of retinal detachment.

All patients with Marfan syndrome need a thorough ophthalmologic evaluation that includes a slit-lamp examination. Biomicroscopy can show regional anterior displacement of the iris or shallowing of the anterior chamber. If the iris surface contour bulges, it may indicate a loss of zonules with vitreous pushing up from behind.

To assess lens stability, instruct the patient to look up, down, right, and left. Tremulousness of the lens (phacodonesis) indicates that it is not well attached. This condition increases the complexity of cataract surgery.

This patient underwent pars plana lensectomy with placement of a posterior chamber intraocular lens, which was secured with sulcus fixation sutures. Her vision improved, and she was able to obtain a driver's license.