The Visual Prognosis in Marfan Syndrome with Ectopia Lentis

Yang, Ji Wook; Kim, Hyun Seung; Lee, Young Chun

Abstract

PURPOSE: To assess the clinical characteristics, indications of operation, operative methods and visual prognosis in Marfan syndrome with ectopia lentis.
METHODS
Medical records of 13 patients (24 eyes) operated due to ectopia lentis were retrospectively analyzed. We evaluated family history, chief complaints, refraction, intraocular lens power, preoperative and postoperative visual acuity, visual prognosis according to the operative methods using one way ANOVA statistically, and postoperative complications.
RESULTS
The range of age at the time of operation was 5~51 years old (mean age 19.4 years old). 6 men, 7 women, 9 had family history. Decreased visual acuity (8 patients) was the main cause to visit hospital, and the knitting the brow (4), the discovery during routine ocular examination (3), strabismus (2), the difficulty in near work (2), monoocular diplopia (1). The ranges of preoperative best corrected visual acuity were 0.01 to 0.3. Postoperative visual acuity was 0.15 to 0.9. In all cases visual acuities increased. High myopia (more than .6 diopter) was seen in 13 eyes, but the ranges of the axial length were normal. The operation was performed with pas plana approach in 7 eyes, by extracapsular cataract extraction in 12 eyes, and by intracapsular cataract extraction in 5 eyes. Intraocular lenses were inserted in 16 eyes. No statistically differences were seen in postoperative best-corrected visual acuity. Preretinal hemorrhages was seen in only one eye postoperatively, soon disappeared and other complications was not seen.
CONCLUSIONS
The most common cause of operation in ectopia lentis was the decreased visual acuity. The cause of high myopia in ectopia lentis was spherophakia due to zonulysis, not axial myopia. The visual prognosis was good in all patients, and no differences was seen respective of operation methods.