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Vascular Ehlers-Danlos Syndrome: Imaging Findings
American Journal of Roentgenology, 2007Vascular Ehlers-Danlos syndrome (EDS), formerly known as EDS type IV, is an autosomal dominant disorder characterized by fragility of medium and large arteries due to type III procollagen deficiency. Our purpose was to review the imaging findings in a cohort of patients with a diagnosis of vascular EDS.The radiologic, surgical, and genetic databases at
Gustavo S Oderich +2 more
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Concurrent Splenic Peliosis and Vascular Ehlers-Danlos Syndrome
Annals of Vascular Surgery, 2009 This case report describes concurrent splenic peliosis and vascular Ehlers-Danlos syndrome (EDS) in a 59-year-old male patient. After splenic rupture due to peliosis, the complicated postoperative period hinted at the possibility of vascular EDS. This diagnosis was confirmed by genetic testing, which revealed a novel point mutation in the COL3A1 gene ...
Bon, A.C. van +3 more
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Vascular Complications of Ehlers-Danlos Syndrome: CT Findings
American Journal of Roentgenology, 2012Patients with vascular Ehlers-Danlos syndrome are predisposed to premature vascular complications. The objective of this article is to review the CT findings and current thoughts about the management of vascular pathologic abnormalities and diseases in patients with Ehlers-Danlos syndrome.The manifestation of vascular Ehlers-Danlos syndrome frequently ...
Linda Chu +2 more
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Spontaneous Splenic Rupture in Vascular Ehlers-Danlos Syndrome
Vascular and Endovascular Surgery, 2015Vascular Ehlers-Danlos Syndrome (VEDS) is a rare autosomal dominant collagen vascular disorder. Different from other Ehler-Danlos Syndrome subtypes, VEDS has poor prognosis due to severe fragility of connective tissues and association with life-threatening vascular and gastrointestinal complications.
Lee Kirksey, Heather Gornik
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Vascular Ehlers–Danlos syndrome
Annales de Génétique, 2004Vascular Ehlers-Danlos syndrome, also known as Ehlers-Danlos syndrome type IV, is a life-threatening inherited disorder of connective tissue, resulting from mutations in the COL3A1 gene coding for type III procollagen. Vascular EDS causes severe fragility of connective tissues with arterial and gastrointestinal rupture, and complications of surgical ...
Dominique P, Germain +1 more
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The dysmorphic phenotype in vascular Ehlers Danlos syndrome
Clinical Dysmorphology, 2022The Ehlers Danlos syndromes are identified by their connective tissue features and are not rich in dysmorphic handles. Vascular Ehlers Danlos syndrome (vEDS) however, is characterised by a recognisable phenotypic constellation of internal and external dysmorphology.
James R, Lyness, Patrick J, Morrison
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Haemothorax in vascular Ehlers-Danlos syndrome
Reumatología Clínica (English Edition), 2019Vascular Ehlers-Danlos syndrome (EDS IV) is a rare genetic disorder characterized by an alteration in the COL3A1 gene which encodes type III collagen. It is the most common type of collagen in vessels of medium size and certain organs such as the intestines and the uterus.
Kevin, Álvarez +2 more
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The vascular ehlers-danlos syndrome
Current Treatment Options in Cardiovascular Medicine, 2006Vascular Ehlers-Danlos syndrome (EDS) is a life-threatening inherited disorder of connective tissue causing severe arterial and gastrointestinal fragility and rupture, as well as complications of surgical and radiologic interventions. The diagnosis should be considered in patients under the age of 45 years who present with arterial tearing or ...
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