4/7/2023 0 Comments Ectopic pupil![]() ![]() ![]() Trans-scleral suturing of the intraocular lens, concurrently with lensectomy, has been described in some patients with ectopia lentis (Omulecki et al. Pars plana lensectomy in both eyes, followed by aphakic correction with glasses or contact lenses, is a standard strategy for surgical intervention in children with ectopia lentis (Halpert and BenEzra 1996 Koenig and Mieler 1996 Anteby et al. In contrast, surgical methods for lensectomy have been well described in the literature. Two apparent surgical indications for ectopia lentis at the textbook level are: (1) lens positioning with the lens edge bisecting the pupil, which makes impossible the optical correction of either the aphakic part or phakic part of the pupil, and (2) anterior displacement of the lens which causes secondary glaucoma. Under the circumstances, there is no established standard of care, described in textbooks or the literature, as to how far observation would be allowed in children with ectopia lentis. The extent of ectopia lentis and its change in the time course vary from patient to patient. Genetic testing has been evolving in ectopia lentis (Chandra and Charteris 2014). The isolated condition of ectopia lentis is either sporadic or familial, and the familial form usually shows an autosomal dominant trait. Another conspicuous feature in ectopia lentis is lens coloboma with the decreased number of ciliary zonules.Įctopia lentis occurs either in isolation or as a syndrome, including Marfan syndrome, Weill–Marchesani syndrome, and homocystinuria (Halpert and BenEzra 1996 Koenig and Mieler 1996). The small lens size in ectopia lentis is in marked contrast with the normal size of the lens in secondary lens subluxation, caused by blunt eye injury. In fact, the ectopic lens is hypoplastic by nature with its diameter smaller relative to the diameter of the ciliary body circumference, and thus, the lens appears to be located eccentrically. The center of the lens is not in alignment with the optical axis of the eyeball, and the equator of the lens with sparse ciliary zonules is visible in one or two quadrants under mydriasis and frequently even through the pupil in the normal size. The visual acuity at near viewing, 0.4 or better, would give a benchmark for continuing observation in children with ectopia lentis.Įctopia lentis is a congenital disorder in children to show malposition of the lens (Halpert and BenEzra 1996 Koenig and Mieler 1996). In conclusions, observation was continued in children with ectopia lentis who had good visual acuity at near viewing. Two patients at the age of 16 and 36 years, additionally, underwent intraocular lens-suturing in both eyes. Lensectomy, combined with anterior vitrectomy, was done from two limbal side ports with a 25-gauge infusion cannula and vitreous cutter. One adult patient developed cataract in ectopic lenses. 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. 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. 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. The diagnoses were Marfan syndrome in six patients, familial ectopia lentis in six, and sporadic ectopia lentis in three. 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 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. Surgical timing for ectopia lentis has not been well described until now. ![]()
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