Arterial complications of vascular Ehlers-Danlos syndrome

Slides:

Advertisements

Similar presentations

Complex aortic and bilateral renal artery aneurysm repair in a young patient with multiple arterial aneurysm syndrome Carlos A. Hinojosa, MD, MSc, Javier.

Advertisements

Endovascular treatment of renal artery aneurysms and renal arteriovenous fistulas Zhongming Zhang, MD, Min Yang, MD, Li Song, MD, Xiaoqiang Tong, MD,

Jordan Knepper, MD, Enrique Criado, MD Journal of Vascular Surgery

A patient with multiple paradoxical emboli

Outcomes of total percutaneous endovascular aortic repair for thoracic, fenestrated, and branched endografts Leonardo R. de Souza, MD, Gustavo S. Oderich,

Type II endoleaks Journal of Vascular Surgery

Reply Journal of Vascular Surgery

Surgeon-modified fenestrated-branched stent grafts to treat emergently ruptured and symptomatic complex aortic aneurysms in high-risk patients Joseph.

Endovascular techniques for arch vessel reconstruction

Chris Burke, MD, Sherene Shalhub, MD, MPH, Benjamin W. Starnes, MD

J. Parfitt, MB, BS, R.T.A. Chalmers, MD, FRCS, J.H.N. Wolfe, MS, FRCS

John Westley Ohman, BS, Kristofer M

Visceral vessel relocation techniques

A percutaneous arterial closure protocol can decrease complications after endovascular interventions in vascular surgery patients Philip P. Goodney,

Aortic dissection: Perspectives in the era of stent-graft repair

Updated strategies to treat acute arterial complications associated with total knee and hip arthroplasty Douglas A. Troutman, DO, Matthew J. Dougherty,

Toronto PowerWire fenestration technique to access false lumen branches in fenestrated endovascular aneurysm repair for chronic type B dissection Steffan.

Endovascular repair of thoracoabdominal aortic aneurysm using the off-the-shelf multibranched t-Branch stent graft Bernardo C. Mendes, MD, Gustavo S.

Late graft explants in endovascular aneurysm repair

Successful coil embolization for rupture of the subclavian artery associated with Ehlers- Danlos syndrome type IV Yasunori Iida, MD, PhD, Yukio Obitsu,

Leopoldo Marine, MD, Rishi Gupta, MD, Heather L. Gornik, MD, Vikram S

Benjamin S. Brooke, MD, George Arnaoutakis, MD, Nazli B

Molecular diagnosis in vascular Ehlers-Danlos syndrome predicts pattern of arterial involvement and outcomes Sherene Shalhub, MD, MPH, James H. Black,

Direct sonographic-guided superior gluteal artery access for treatment of a previously treated expanding internal iliac artery aneurysm Michael M. Herskowitz,

Complex aortic and bilateral renal artery aneurysm repair in a young patient with multiple arterial aneurysm syndrome Carlos A. Hinojosa, MD, MSc, Javier.

Endovascular repair of tortuous recurrent femoral-popliteal aneurysm in a patient with Loeys-Dietz syndrome Sophie Wang, BS, Amber Kernodle, MD, MPH,

Thomas S. Maldonado, MD, Caron B

Endovascular treatment of aneurysms using fenestrated-branched endografts with distal inverted iliac limbs Vikalp Jain, MD, Peter Banga, MD, Raghuveer.

Intimal angiosarcoma presenting with common femoral artery aneurysm

Gary R. Seabrook, MD Journal of Vascular Surgery

Lower extremity compartment syndrome after elective percutaneous fenestrated endovascular repair of an abdominal aortic aneurysm John F. Charitable,

Martijn L. Dijkstra, BA, Matthew J. Eagleton, MD, Roy K

Thoracic endovascular aortic repair of aortobronchial fistulas

Late conversion after endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome Georgios Karaolanis, MD, MSc, PhD, Özgun.

Raghuveer Vallabhaneni, MD, Luis A. Sanchez, MD

Niyant V. Patel, MD, Graham W. Long, MD, Zulfiqar F

Nanette R. Reed, MD, Gustavo S

Michael R. Corey, MD, Emel A. Ergul, MS, Richard P

Fenestrated and branched endovascular aneurysm repair outcomes for type II and III thoracoabdominal aortic aneurysms Matthew J. Eagleton, MD, Matthew.

Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome Maseer A. Bade, MD, Luis A. Queral, MD, Dipankar Mukherjee, MD,

Complex aortic and bilateral renal artery aneurysm repair in a young patient with multiple arterial aneurysm syndrome Carlos A. Hinojosa, MD, MSc, Javier.

Endovascular repair of an iliac artery aneurysm in a patient with Ehlers-Danlos syndrome type IV Britt H. Tonnessen, MD, W. Charles Sternbergh, MD, Krishna.

Ultrasound-guided percutaneous endovascular aneurysm repair success is predicted by access vessel diameter Rodney P. Bensley, MD, Rob Hurks, MD, Zhen.

Endovascular repair of a celiac trunk aneurysm with a new multilayer stent Giampaolo Carrafiello, MD, Nicola Rivolta, MD, Matteo Annoni, MD, Federico.

Jonathan J. Sexton, MD, David R. Whittaker, MD

Mitchell W. Cox, MD, David R

Successful endovascular repair of an aneurysm of the ductus diverticulum with a branched stent graft: Case report and review of literature Naritatsu.

Stylocarotid artery syndrome

Thomas J. Ward, MD, Michael L. Marin, MD, Robert A. Lookstein, MD

Amplatzer Vascular Plug to occlude the internal iliac arteries in patients undergoing aortoiliac aneurysm repair Chi D. Ha, MD, David Calcagno, MD Journal.

Management of aneurysms involving branches of the celiac and superior mesenteric arteries: A comparison of surgical and endovascular therapy Ulka Sachdev,

Reply Journal of Vascular Surgery

Endovascular repair of a giant renal vein aneurysm arising from an arteriovenous fistula Rajanshu Verma, MD, Anthony King, MD, Alex Sasani, MD, Vishal.

Three-dimensional fusion computed tomography decreases radiation exposure, procedure time, and contrast use during fenestrated endovascular aortic repair

Femoral pseudoaneurysms after percutaneous access

Endovascular treatment of renal artery aneurysms and renal arteriovenous fistulas Zhongming Zhang, MD, Min Yang, MD, Li Song, MD, Xiaoqiang Tong, MD,

Successful percutaneous embolization of a symptomatic celiac artery dissection with aneurysmal dilation with detachable vascular plugs Michel Batt, MD,

Endovascular management of superior mesenteric artery pseudoaneurysm

J. Parfitt, MB, BS, R.T.A. Chalmers, MD, FRCS, J.H.N. Wolfe, MS, FRCS

Boonprasit Kritpracha, MD, Anthony J. Comerota, MD,

Endovascular repair of a penetrating thoracic aortic ulcer by way of the carotid artery Michael J Heidenreich, MD, David G Neschis, MD, Michael J Costanza,

H. Edward Garrett, MD, Robert W. Heidepriem, MD, L. Paul Broadbent, MD

Regarding “Vascular abnormalities in patients with neurofibromatosis syndrome type I: Clinical spectrum, management, and results” John B. Chang, MD,

Rebecca Jeanmonod, MD, Chad Lewis, MD Journal of Vascular Surgery

Nanette R. Reed, MD, Gustavo S

Joshua Sylvan, Roy K. Greenberg, MD, Tara M. Mastracci, MD, Matthew J

Ronald M. Fairman, MD, Lorraine Nolte, PhD, Scott A

Are intrasac pressure measurements useful after endovascular repair of abdominal aortic aneurysms? Ross Milner, MD, Paola De Rango, MD, Fabio Verzini,

Late conversion after endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome Georgios Karaolanis, MD, MSc, PhD, Özgun.

Combined transbrachial and transfemoral strategy to deploy an iliac branch endoprosthesis in the setting of a pre-existing endovascular aortic aneurysm.

Presentation transcript:

Arterial complications of vascular Ehlers-Danlos syndrome Matthew J. Eagleton, MD Journal of Vascular Surgery Volume 64, Issue 6, Pages 1869-1880 (December 2016) DOI: 10.1016/j.jvs.2016.06.120 Copyright © 2016 Society for Vascular Surgery Terms and Conditions

Fig 1 Basic steps of type III collagen production. A, Type III collagen is coded for on the long arm of chromosome 2 by gene COL3A1 located at position 2q31 (arrow). Defects in this gene location account for the development of vascular Ehlers-Danlos syndrome (EDS). B, The gene undergoes transcription and translation producing the pro-α1 (III) proteins. A unique feature of these proteins is the presence of a glycine residue in every third position of the polypeptide chains resulting in a (Gly-X-Y)n repeat structure. C, This positioning allows for assembly into a densely packed triple helix formation when three of the α-chains combine to form a homotrimer. The protein is further modified, packaged and secreted through the cell's Golgi apparatus. Ultimately, N-terminal and C-terminal ends are cleaved creating a mature protein. D, In the extracellular space, cross-linking occurs between homotrimers creating a stable collagen fibril. Journal of Vascular Surgery 2016 64, 1869-1880DOI: (10.1016/j.jvs.2016.06.120) Copyright © 2016 Society for Vascular Surgery Terms and Conditions

Fig 2 Spectrum of facial features in individuals with vascular Ehlers-Danlos syndrome (EDS) shows variability among patients and does not necessarily correlate with the severity of the underlying arterial pathology. These four individuals died of vascular complications. A, Caucasian man (MIN mutation, c.2553+1delG) presenting with characteristic vascular EDS facies, including proptotic eyes, long and thin nose, minimal subcutaneous facial fat, and a triangular-shaped face. B, Hispanic woman (MIN mutation, c.3545G>A p.G1182E) with mildly proptotic eyes, a long thin nose, and a hypotrophic forehead scar, but otherwise normal facial features. C, Caucasian man (MIN mutation, c.2870G>T, p.G957) with downslanting palpebral fissures, long thin nose, thin lips, and attached pinna. D, Caucasian woman (c.665G>T, pG222V) presenting with a long thin nose but otherwise normal facial features. Written consent for publication was obtained from the original authors. MIN, mutations that lead to minimal (10-15%) normal type III collagen production. (Reproduced with permission from Elsevier from Shalhub et al. J Vasc Surg 2014;60:160-9.) Journal of Vascular Surgery 2016 64, 1869-1880DOI: (10.1016/j.jvs.2016.06.120) Copyright © 2016 Society for Vascular Surgery Terms and Conditions

Fig 3 Vascular Ehlers-Danlos syndrome (EDS) in a patient treated for a large hepatic artery aneurysm. A and B, The patient was identified as having an expanding hepatic artery aneurysm (arrows) and an associated small left renal artery aneurysm (arrowhead). The hepatic aneurysm had reach 3.7 cm in diameter. C and D, The patient underwent endovascular coil embolization of the branches arising from the aneurysm and the aneurysm sac itself. This was performed through a cutdown on the left brachial artery, which at the end of the procedure was repaired with pledgetted sutures. Follow-up imaging demonstrated exclusion of the hepatic aneurysm. Journal of Vascular Surgery 2016 64, 1869-1880DOI: (10.1016/j.jvs.2016.06.120) Copyright © 2016 Society for Vascular Surgery Terms and Conditions

Fig 4 Technique for management of the access vessel in fragile vasculature of Ehlers-Danlos syndrome (EDS) patient. A, A “U” stitch monofilament suture is placed with a pledget buttress, and needle entry is made within the area. B, Sheath access is obtained and manipulated or exchanged minimally to avoid femoral tears. C, The pledgetted “U” stitch suture is tied down as sheath is removed. D, Circumferential felt reinforcement is approximated to reduce systolic pulse wave stretch on sutures and to prevent late pseudoaneurysm formation. (Reproduced with permission from Elsevier from Brook BS, et al. J Vasc Surg 2010;51:131-9.) Journal of Vascular Surgery 2016 64, 1869-1880DOI: (10.1016/j.jvs.2016.06.120) Copyright © 2016 Society for Vascular Surgery Terms and Conditions