How far is observation allowed in patients with ectopia lentis?

Surgical timing for ectopia lentis has not been well described until now. The purpose of this study is to find a benchmark as to how far observation would be allowed in children with ectopia lentis when they and their families are reluctant to go through surgery. Retrospective review was made on 15 consecutive patients (14 children and one adult) with ectopia lentis in both eyes, seen at a referral-based institution in 5 years from April 2008 to March 2013, to survey the reasons for continuing observation or deciding surgical intervention. The diagnoses were Marfan syndrome in six patients, familial ectopia lentis in six, and sporadic ectopia lentis in three. Observation was continued in nine patients with the age at the final visit, ranging from 4 to 17 (median 9) years, because six children had good visual acuity at both near and distant viewing with glasses, and three children had visual acuity of 0.4 at near viewing despites poor visual acuity at distant viewing with glasses. In contrast, lensectomy was determined in six patients (5 children and one adult) with the age at surgery, ranging from 4 to 36 (median 9) years, and the age at the final visit, ranging from 7 to 42 (median 11) years, mainly because of poor visual acuity at near and distant viewing. More specific causes for surgeries in five children were the optical axis to become aphakic due to the progression of ectopia in the course in two children, lens dislocation to the anterior chamber after blunt eye injury in one child, and difficulty in studying at school classes in two children. One adult patient developed cataract in ectopic lenses. Lensectomy, combined with anterior vitrectomy, was done from two limbal side ports with a 25-gauge infusion cannula and vitreous cutter. Two patients at the age of 16 and 36 years, additionally, underwent intraocular lens-suturing in both eyes. In conclusions, observation was continued in children with ectopia lentis who had good visual acuity at near viewing. The visual acuity at near viewing, 0.4 or better, would give a benchmark for continuing observation in children with ectopia lentis.

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