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Corneal Abnormalities in Ehlers-Danlos Syndrome Type VI

 

作者: James Cameron,  

 

期刊: Cornea  (OVID Available online 1993)
卷期: Volume 12, issue 1  

页码: 54-59

 

ISSN:0277-3740

 

年代: 1993

 

出版商: OVID

 

关键词: Blue sclera;Ehlers-Danlos syndrome;Corneal rupture;Sclerocornea;Hydrops;Curvature abnormalities

 

数据来源: OVID

 

摘要:

Eleven patients with blue sclera, limbus-to-limbus corneal thinning, hypermobile joints, and consanguineous parents were examined between January 1983 and September 1991. The clinical diagnosis was consistent with the Ehlers-Danlos syndrome type VI phenotype in all patients. A "halo" sign at the limbus was present in all patients. Corneal rupture occurred in seven patients (nine eyes) either spontaneously or following minimal trauma. Acute hydrops occurred in three patients. Bilateral microcornea was present in one patient and two patients had a unilateral increased corneal diameter as a result of secondary glaucoma after trauma. Peripheral sclerocornea was present bilaterally in five patients. Curvature abnormalities included cornea plana, keratoconus, and keratoglobus.

 

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