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Endovascular Treatment of Spontaneous Carotid-Cavernous Fistula in Ehlers-Danlos Syndrome Type IV

 

作者: Maurizio Cesari,   Gian Paolo Rossi,   Katharine M. Dyne,   Daniele Fiore,   Achille C. Pessina,  

 

期刊: Cerebrovascular Diseases  （Karger Available online 1997）
卷期: Volume 7, issue 5  

页码: 273-279

 

ISSN:1015-9770

 

年代: 1997

 

DOI:10.1159/000108208

 

出版商: S. Karger AG

 

关键词: Ehlers-Danlos syndrome;Carotid-cavernous fistula;Type-III collagen;Treatment;endovascular

 

数据来源: Karger

 

摘要:

We report a sporadic case of type-IV Ehlers-Danlos Syndrome (EDS) in a woman presenting with a spontaneous carotid-cavernous fistula. This complication was treated successfully by endovascular treatment (EVT) with long-term relief. Analysis of collagens synthesized by the patient''s dermal fibroblasts in culture showed impaired secretion of abnormally slow, electrophoretically migrating type-III collagen, suggesting an underlying defect in the COL3A1 gene. The clinical features of type IV EDS and the outcome of EVT are reviewed.

 

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