Surgery of ectopia lentis

Abstract

Purpose Ectopia lentis is a patology with lens dislocation presumably secondary to zonular fiber weakness. Ectopia lentis can occur in isolation, in association with other ocular disorders or as part of systemic disorder. Marfan´s syndrome and homocystinuria are the most frequent cause of heritable ectopia lentis. Marfan syndrome (MFS) is a hereditary connective tissue disorder. Studies of MFS have established the critical contribution of fibrillin‐1 deficiency to disease progression through altered cell‐matrix interactions and dysregulated TGF‐β signalling. Methods We report four eyes of 4 children aged from 2 years to 4 years with ectopia lentis Both cases showed bilateral and symmetric lens dislocation with low visual acuity. One of the children was diagnosed of Marfan syndrome. Results We practised Twenty three gauge two port pars plana lensectomy without intraocular lenses. Conclusion Pars plana lensectomy is a safe, effective procedure for the management of ectopia lentis. Earlier surgery is indicated to prevent amblyopia and improve visual acuity. Marfan syndrome, homocystinuria, trauma and simple ectopia lentis are the most common caused of pediatric lens subluxation. Ectopia lentis in children continues to be a diagnostic and therapeutic challenge for ophthalmologists. The convencional surgical management of congenital subluxated lenses infrequently associated with a high incidence of complications leading to poor visual prognosis. Surgical intervention is necessary when lens subluxation causes a significant refractive error resulting in amblyopia.