Ten‐year reinvestigation of ocular manifestations in Marfan syndrome

Abstract

Importance: Long-term follow-up of Marfan syndrome (MFS) patients. Background: Investigate changes in ocular features in MFS patients fulfilling the Ghent-2 criteria following a period of 10 years. Design: Repeated cross-sectional study with two observations. Participants: Eighty-four MFS patients were investigated in 2003-2004 (baseline). Forty-four of these patients (52%) were examined after 10 years. Methods: A comprehensive ocular examination performed at baseline and follow-up. Main Outcome Measures: Development or progression of ectopia lentis (EL).Results: At follow-up, mean age was 50.1 AE 11.9 years (range: 30-80 years), 74% were female and 70% of the patients were diagnosed with EL compared to 66% at baseline. Two patients (3 eyes) had developed EL over the decade, representing a 13% risk. Furthermore, one eye had progressed from a subtle tilt of the lens to dislocation. We found no significant change in the axial length (P = 0.96), the corneal curvature (P = 0.64) or the spherical equivalent (P = 0.23). Best corrected visual acuity was improved at follow-up (P = 0.02). There were 7% and 33% risks for development of retinal detachment and cataract between baseline and follow-up, respectively. Conclusions and Relevance: Our study indicates that even though EL typically occurs at an early stage in most MFS patients, there is still a risk of developing EL in adulthood. The risk of developing vision-threatening complications such as retinal detachment and cataract was much higher than in the normal population, but even so, the visual potential of the MFS patients was relatively good. K E Y W O R D Saxial length, corneal curvature, ectopia lentis, Marfan syndrome, visual acuity

Long-Term Suture Breakage After Scleral Fixation of a Modified Capsular Tension Ring with Polypropylene 10-0 Suture

“…Ectopia lentis is usually seen from childhood, but it can also progress in adults. 27 , 28 It often requires surgical treatment, and since the zonules are weakened, suture-fixated MCTR is usually recommended, if possible to retain the lens capsule for in-the-bag IOL placement. 20 In the eyes of the Marfan patients in our study, the suture breakage rate was 42%.…”

Analysis of axial Length of Marfan Syndrome with Bilateral Ectopia Lentis by Z-scores for Chinese Patients under 20 years Old

“…The conclusions should be viewed with caution for other ethnic populations. Third, adult patients were excluded from the current study, as AL were shown to be stable in adults (> 30 years old) with MFS during a 10-year follow up 37 . The cross-sectional nature of our study also limited our ability to track changes in AL data over time.…”

Cataract and systemic disease: A review

“…The main organ systems affected are the cardiovascular, skeletal and ocular systems. Ocular findings include myopia, ectopia lentis, retinal detachment, glaucoma and early cataracts 77,78 . Ectopia lentis is the primary ocular complication of Marfan syndrome and occurs in approximately 60% of cases 77 .…”

“…Ocular findings include myopia, ectopia lentis, retinal detachment, glaucoma and early cataracts. 77,78 Ectopia lentis is the primary ocular complication of Marfan syndrome and occurs in approximately 60% of cases. 77 Though the overall prevalence of cataracts is not higher in Marfan syndrome, they tend to present at a younger age.…”