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

2. 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  , DOI: ( /j.jvs )
Copyright © 2016 Society for Vascular Surgery Terms and Conditions

3. 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 delG) 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  , DOI: ( /j.jvs )
Copyright © 2016 Society for Vascular Surgery Terms and Conditions

4. 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  , DOI: ( /j.jvs )
Copyright © 2016 Society for Vascular Surgery Terms and Conditions

5. 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  , DOI: ( /j.jvs )
Copyright © 2016 Society for Vascular Surgery Terms and Conditions