A descriptive study of ocular characteristics in Marfan syndrome

被引:63
作者
Konradsen, Tiina R. [1 ,2 ]
Zetterstrom, Charlotta [2 ]
机构
[1] St Erik Eye Hosp, Dept Anterior Segment, S-11282 Stockholm, Sweden
[2] Karolinska Inst, Dept Clin Neurosci, Stockholm, Sweden
关键词
aphakia; axial length; cataract; ectopia lentis; flattened cornea; Marfan syndrome; myopia; pseudophakia; retinal detachment; NOSOLOGY; ORBSCAN; FBN1;
D O I
10.1111/aos.12068
中图分类号
R77 [眼科学];
学科分类号
100212 ;
摘要
Purpose: To study the ocular characteristics in patients with Marfan syndrome (MFS). Methods: One hundred and two eyes of 51 participants with MFS were included in this descriptive study. Ocular characteristics, visual acuity (VA) and biometric values were studied. Results: The mean patient age was 39.0 years (range, 12.7-71.6). Seventy-seven eyes were phakic, five aphakic and 20 pseudophakic. Thirty-one eyes had ectopia lentis (EL), 12 eyes cataract, nine eyes strabismus, five eyes glaucoma and four eyes had undergone surgery for a retinal detachment (RD). Ninety-four eyes (92%) had a best spectacle-corrected VA of 0.3 logarithm of the minimum angle of resolution (logMAR) or better. In the 77 phakic eyes, myopia exceeding -3 D was seen in 12 /31 eyes (39%) with EL, and in 12 /46 eyes (26%) without EL. The mean axial length (AL) was 24.73 mm (range, 21.59-32.75); 39 eyes (51%) had abnormally increased AL (>= 24.5 mm). The mean K-med (mean value of K-min and K-max) was 42.2 D (38.7-46.9 D). No significant difference was found in refraction, AL or corneal curvature between eyes with and without EL. Lens-related astigmatism was seen in 15 (48%) eyes with EL versus four (12%) eyes without EL. Seventeen of the pseudophakic eyes underwent surgery for EL, with history of postoperative RD in one eye. Conclusion: Despite increased AL, high myopia is not as common in MFS as expected because of a flat cornea. Biometry should be considered in suspected cases of MFS when EL is not present.
引用
收藏
页码:751 / 755
页数:5
相关论文
共 19 条
[1]   Comparison of Clinical Presentations and Outcomes Between Patients With TGFBR2 and FBN1 Mutations in Marfan Syndrome and Related Disorders [J].
Attias, David ;
Stheneur, Chantal ;
Roy, Carine ;
Collod-Beroud, Gwenaelle ;
Detaint, Delphine ;
Faivre, Laurence ;
Delrue, Marie-Ange ;
Cohen, Laurence ;
Francannet, Christine ;
Beroud, Christophe ;
Claustres, Mireille ;
Iserin, Franck ;
Van Kien, Philippe Khau ;
Lacombe, Didier ;
Le Merrer, Martine ;
Lyonnet, Stanislas ;
Odent, Sylvie ;
Plauchu, Henri ;
Rio, Marlene ;
Rossi, Annick ;
Sidi, Daniel ;
Steg, Philippe Gabriel ;
Ravaud, Philippe ;
Boileau, Catherine ;
Jondeau, Guillaume .
CIRCULATION, 2009, 120 (25) :2541-2549
[2]   One million cataract surgeries: Swedish National Cataract Register 1992-2009 [J].
Behndig, Anders ;
Montan, Per ;
Stenevi, Ulf ;
Kugelberg, Maria ;
Lundstrom, Mats .
JOURNAL OF CATARACT AND REFRACTIVE SURGERY, 2011, 37 (08) :1539-1545
[3]   INTERNATIONAL NOSOLOGY OF HERITABLE DISORDERS OF CONNECTIVE-TISSUE, BERLIN, 1986 [J].
BEIGHTON, P ;
DEPAEPE, A ;
DANKS, D ;
FINIDORI, G ;
GEDDEDAHL, T ;
GOODMAN, R ;
HALL, JG ;
HOLLISTER, DW ;
HORTON, W ;
MCKUSICK, VA ;
OPITZ, JM ;
POPE, FM ;
PYERITZ, RE ;
RIMOIN, DL ;
SILLENCE, D ;
SPRANGER, JW ;
THOMPSON, E ;
TSIPOURAS, P ;
VILJOEN, D ;
WINSHIP, I ;
YOUNG, I .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1988, 29 (03) :581-594
[4]   Accuracy of Orbscan II slit-scanning elevation topography [J].
Cairns, G ;
McGhee, CNJ ;
Collins, MJ ;
Owens, H ;
Gamble, GD .
JOURNAL OF CATARACT AND REFRACTIVE SURGERY, 2002, 28 (12) :2181-2187
[5]  
DePaepe A, 1996, AM J MED GENET, V62, P417, DOI 10.1002/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO
[6]  
2-R
[7]   Distribution of Axial Length and Ocular Biometry Measured Using Partial Coherence Laser Interferometry (IOL Master) in an Older White Population [J].
Fotedar, Reena ;
Wang, Jie Jin ;
Burlutsky, George ;
Morgan, Ian G. ;
Rose, Kathryn ;
Wong, Tien Y. ;
Mitchell, Paul .
OPHTHALMOLOGY, 2010, 117 (03) :417-423
[8]  
Gonzalez Perez Javier, 2004, Eye Contact Lens, V30, P74
[9]   ASCERTAINMENT AND SEVERITY OF MARFAN-SYNDROME IN A SCOTTISH POPULATION [J].
GRAY, JR ;
BRIDGES, AB ;
FAED, MJW ;
PRINGLE, T ;
BAINES, P ;
DEAN, J ;
BOXER, M .
JOURNAL OF MEDICAL GENETICS, 1994, 31 (01) :51-54
[10]  
Judge DP, 2005, LANCET, V366, P1965, DOI 10.1016/S0140-6736(05)67789-6