Vascular complications of Ehlers-Danlos syndrome: CT findings.

OBJECTIVE Patients 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. CONCLUSION The manifestation of vascular Ehlers-Danlos syndrome frequently involves multiple vascular segments, with aneurysms and dissections being the most common imaging findings. The traditional practice of avoiding surgery until complications develop is being modified in select cases.

[1]  S. Nampoothiri,et al.  Imaging findings in a distinct lethal inherited arteriopathy syndrome associated with a novel mutation in the FBLN4 gene , 2014, European Radiology.

[2]  Jeffrey T. Krawiec,et al.  Elastin and collagen fibre microstructure of the human aorta in ageing and disease: a review , 2013, Journal of The Royal Society Interface.

[3]  C. Bruce,et al.  CT and MRI assessment of the aortic root and ascending aorta. , 2013, AJR. American journal of roentgenology.

[4]  S. Laurent,et al.  Effect of Celiprolol on Prevention of Cardiovascular Events in Vascular Ehlers-Danlos Syndrome , 2009 .

[5]  P. Byers,et al.  Successful endovascular repair of acute type B aortic dissection in undiagnosed Ehlers-Danlos syndrome type IV. , 2009, European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery.

[6]  J. Black,et al.  Contemporary management of vascular complications associated with Ehlers-Danlos syndrome. , 2009, Journal of vascular surgery.

[7]  M. Schepens.  Re: “Thoracic Aortic Endografting in Patients with Connective Tissue Disease” , 2008, Journal of endovascular therapy : an official journal of the International Society of Endovascular Specialists.

[8]  T. Vrtiska,et al.  Vascular Ehlers-Danlos syndrome: imaging findings. , 2007, AJR. American journal of roentgenology.

[9]  L. Queral,et al.  Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. , 2007, Journal of vascular surgery.

[10]  P. Byers,et al.  Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. , 2000, The New England journal of medicine.

[11]  D. Germain.  The vascular ehlers-danlos syndrome , 1999, Current treatment options in cardiovascular medicine.

[12]  P. Beighton,et al.  Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). , 1998, American journal of medical genetics.

[13]  J. Miles,et al.  Spontaneous arterial perforation: the Ehlers-Danlos specter. , 1987, Journal of vascular surgery.

[14]  A. Gomes,et al.  Perforation of the superior vena cava: a complication of digital angiography in Ehlers-Danlos syndrome. , 1984, AJR. American journal of roentgenology.

[15]  V. McKusick,et al.  Patients with Ehlers-Danlos syndrome type IV lack type III collagen. , 1975, Proceedings of the National Academy of Sciences of the United States of America.

[16]  A. Barabas.  Heterogeneity of the Ehlers-Danlos syndrome: description of three clinical types and a hypothesis to explain the basic defect(s). , 1967, British medical journal.

[17]  R. S. Mitchell,et al.  Expert consensus document on the treatment of descending thoracic aortic disease using endovascular stent-grafts. , 2008, The Annals of thoracic surgery.

[18]  N. Lindor,et al.  From the Southern Association for Vascular Surgery The spectrum , management and clinical outcome of Ehlers-Danlos syndrome type IV : A 30-year experience , 2005 .