Ten-year reinvestigation of ocular manifestations in Marfan syndrome.

Autor: Sandvik GF; Department of Ophthalmology, Oslo University Hospital, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway., Vanem TT; Institute of Clinical Medicine, University of Oslo, Oslo, Norway., Rand-Hendriksen S; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.; Department of TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesodden, Norway., Cholidis S; Department of Ophthalmology, Oslo University Hospital, Oslo, Norway., Saethre M; Department of Ophthalmology, Oslo University Hospital, Oslo, Norway., Drolsum L; Department of Ophthalmology, Oslo University Hospital, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Jazyk: angličtina
Zdroj: Clinical & experimental ophthalmology [Clin Exp Ophthalmol] 2019 Mar; Vol. 47 (2), pp. 212-218. Date of Electronic Publication: 2018 Nov 11.
DOI: 10.1111/ceo.13408
Abstrakt: 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.
(© 2018 Royal Australian and New Zealand College of Ophthalmologists.)
Databáze: MEDLINE
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