Evaluation of surgical intervention in familial isolated simple ectopia lentis: younger v. older generations

There continues to be a debate whether surgical management of ectopia lentis in children is an appropriate course to improve visual acuity and prevent further amblyopia over medical and optical management. The long term outcome and postoperative status of three generations of patients in a single family with simple ectopia lentis is presented.

Nine family members (ages 6-61 years) were evaluated at Children's Hospital of Michigan (CHM) (6 patients) and Kresge Eye Institute (KEI) (3 patients) for primary or secondary visual acuity problems and a family history of simple bilateral ectopia lentis without any systemic manifestations.

Three of the four children with ectopia lentis had improved postoperative vision OU and one child was moderately amblyopic following lensectomy with 6 years followup. Of the older generation, one of the three adults had removal of dislocated lenses, with 20/50 amblyopia in one eye and 20/25 vision in the other. The other two adults were treated for end stage glaucoma with poor visual acuity.

For the younger generations of this family, surgical intervention for simple ectopia lentis provided improvement in visual acuity. Without surgery, amblyopia may have occurred in one or both eyes. For the oldest generation of this family, glaucoma and poor vision was the end result. Hopefully, the earlier treatment of the ectopia lentis in these children will result in better vision now and in the long term.