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 time, Schirmer I testing, axial length and anterior chamber depth measurement, corneal topography, corneal pachymetry, and confocal microscopy. Main outcome measures included comparing ocular anomalies in JHS/EDS-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 were 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). Pathologic 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. © 2012 Elsevier Inc. All rights reserved.
Ocular features in joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type: A clinical and in vivo confocal microscopy study / Gharbiya, Magda; Moramarco, Antonietta; Castori, Marco; Parisi, Francesco; Claudia, Celletti; Marenco, Marco; Isabella, Mariani; Grammatico, Paola; Filippo, Camerota. - In: AMERICAN JOURNAL OF OPHTHALMOLOGY. - ISSN 0002-9394. - STAMPA. - 154:3(2012), pp. 593-600. [10.1016/j.ajo.2012.03.023]
Ocular features in joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type: A clinical and in vivo confocal microscopy study
GHARBIYA, Magda;MORAMARCO, Antonietta;CASTORI, MARCO;PARISI, FRANCESCO;MARENCO, MARCO;GRAMMATICO, Paola;
2012
Abstract
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 time, Schirmer I testing, axial length and anterior chamber depth measurement, corneal topography, corneal pachymetry, and confocal microscopy. Main outcome measures included comparing ocular anomalies in JHS/EDS-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 were 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). Pathologic 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. © 2012 Elsevier Inc. All rights reserved.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
