Ocular Features in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type: A Clinical and In Vivo Confocal Microscopy Study

被引：37
|
作者：
Gharbiya, Magda [3 ]
Moramarco, Antonietta [3 ]
Castori, Marco [1 ]
Parisi, Francesco [3 ]
Celletti, Claudia [2 ]
Marenco, Marco [3 ]
Mariani, Isabella [3 ]
Grammatico, Paola [1 ]
Camerota, Filippo [2 ]
机构：
[1] Univ Roma La Sapienza, Div Med Genet, Dept Mol Med, San Camilloforlanini Hosp, I-00161 Rome, Italy
[2] Univ Roma La Sapienza, Dept Phys Med & Rehabil, Umberto Hosp 1, I-00161 Rome, Italy
[3] Univ Roma La Sapienza, Dept Ophthalmol, Umberto Hosp 1, I-00161 Rome, Italy
来源：
AMERICAN JOURNAL OF OPHTHALMOLOGY | 2012年 / 154卷 / 03期
关键词：
HEREDITARY DISORDERS; CONNECTIVE-TISSUE; SJOGRENS-SYNDROME; CORNEAL NERVES; COLLAGEN; EYE; POPULATION; DEFICIENCY; MOBILITY; CRITERIA;
D O I：
10.1016/j.ajo.2012.03.023
中图分类号：
R77 [眼科学];
学科分类号：
100212 ;
摘要：
PURPOSE: To investigate ocular anomalies in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT). DESIGN: Prospective, cross-sectional study. METHODS: Forty-four eyes of 22 consecutive patients with an established diagnosis of JHS/EDS-HT and 44 eyes of 22 age- and gender-matched control subjects. Administration of a standardized questionnaire (Ocular Surface Disease Index) and a complete ophthalmologic examination, including assessment of best-corrected visual acuity, slit-lamp biomicroscopy, intraocular pressure measurement, indirect ophthalmoscopy, tear-film break-up tin e, Schirmer I testing, axial length and anterior chamber depth measurement, corneal topography, corneal padhymetry, and confocal microscopy. Main outcome measures included comparing ocular anomalies in JHS/HIS-HT and control eyes. RESULTS: JHS/EDS-HT patients reported dry eye symptoms more commonly than controls (P < .0001). Scores of tear-film break-up time and Schirmer I test we re significantly lower in JHS/EDS-HT eyes (P < .0001). Minor lens opacities were significantly more common in the JHS/EDS-HT group (13.6%; P < .05). Pahologic myopia with abnormal vitreous was found in 7 JHS/EDS-HT eyes (15.9%) and 0 controls (P = .01). Corneas were significantly steeper and the best-fit sphere index was significantly higher in JHS/EDS-HT group (P < .01). By confocal microscopy, the JHS/EDS-HT group showed lower density of cells in the superficial epithelium (P < .001) and higher density of stromal keratocytes in anterior and posterior stroma (P <. 0001). CONCLUSIONS: The most consistent association of eye anomalies in the JHS/EDS-HT group included xerophthalmia, steeper corneas, pathologic myopia, and vitreous abnormalities, as well as a higher rate of minor lens opacities. These findings indicate the need for ophthalmologic survey in the assessment and management of patients with JHS/EDS-HT. (Am J Ophthalmol 2012;154:593-600. (C) 2012 by Elsevier Inc. All rights reserved.)
引用
收藏
页码：593 / 600
页数：8
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