Clinical & experimental ophthalmology · 2018
Ten-year reinvestigation of ocular manifestations in Marfan syndrome.
СтатьяНазвание и аннотация: Перевод в очереди
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Sandvik Gunhild F, Vanem Thy T, Rand-Hendriksen Svend, Cholidis Symira, Saethre Marit, Drolsum Liv
Аннотация
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 ± 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.
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 ± 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.