A descriptive study of ocular characteristics in Marfan syndrome

被引:61
|
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 50 条
  • [21] Marfan Syndrome: A Case Study
    Al-Mulla, Maysah Faisal
    INTERNATIONAL JOURNAL OF SCIENTIFIC STUDY, 2013, 1 (01) : 38 - 47
  • [22] Ocular Involvement and Treatment Pattern in Korean Patients with Marfan Syndrome: A Population-Based Study
    Kim, Seongho
    Han, Kyungdo
    Park, Sanghyun
    Kim, EunAh
    Song, Su Jeong
    OPHTHALMIC EPIDEMIOLOGY, 2024, 31 (02) : 112 - 118
  • [23] Characteristics Of Patients With Spontaneous Pneumothorax In Marfan Syndrome
    Taille, C.
    Tchitchinadze, M.
    Milleron, O.
    Crestani, B.
    Jondeau, G.
    AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2015, 191
  • [24] Pathophysiology and Japanese clinical characteristics in Marfan syndrome
    Fujita, Daishi
    Takeda, Norifumi
    Imai, Yasushi
    Inuzuka, Ryo
    Komuro, Issei
    Hirata, Yasunobu
    PEDIATRICS INTERNATIONAL, 2014, 56 (04) : 484 - 491
  • [25] Marfan Syndrome and Loeys-Dietz Syndrome: Distinguishing Characteristics
    Swenty, Constance F.
    JNP-JOURNAL FOR NURSE PRACTITIONERS, 2015, 11 (10): : 1053 - 1054
  • [26] Marfan syndrome, not Marfan's syndrome
    Cheng, TO
    CIRCULATION, 1999, 99 (01) : 166 - 167
  • [27] Ocular Manifestations and Biometrics in Marfan's Syndrome from Eastern Nepal
    Suwal, Rinkal
    Khadka, Simanta
    Joshi, Purushottam
    CLINICAL OPHTHALMOLOGY, 2020, 14 : 2463 - 2472
  • [28] Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective
    Esfandiari, Hamed
    Ansari, Shabnam
    Mohammad-Rabei, Hossein
    Mets, Marilyn B.
    JOURNAL OF OPHTHALMIC & VISION RESEARCH, 2019, 14 (01) : 71 - 77
  • [29] Marfan syndrome, not Marfan's syndrome -: Response
    Doroshow, RW
    Lin, HJ
    CIRCULATION, 1999, 99 (01) : 167 - 167
  • [30] LINKAGE STUDY OF MARFAN-SYNDROME
    SCHLEUTERMANN, DA
    MURDOCH, JL
    WALKER, BA
    BIAS, WB
    CHASE, GA
    FREIDHOFF, LB
    MCKUSICK, VA
    CLINICAL GENETICS, 1976, 10 (01) : 51 - 53