1. Sonesson B, Hansen F, Lanne T. The mechanical properties of elastic arteries in Ehlers-Danlos syndrome. Eur J Vasc Endovasc Surg. 1997;14:258–264.
2. Bergqvist D. Ehlers-Danlos type IV syndrome. A review from a vascular surgical point of view. Eur J Surg. 1996;162:163–170.
3. Kim HS, Choi CH, Lee TH, et al. Fusiform aneurysm presenting with cervical radiculopathy in Ehlers-Danlos syndrome. J Korean Neurosurg Soc. 2010;48:528–531.
4. Habib K, Memon MA, Reid DA, et al. Spontaneous common iliac arteries rupture in Ehlers-Danlos syndrome type IV: report of two cases and review of the literature. Ann R Coll Surg Engl. 2001;83:96–104.
5. Mandeville K, Bicknell C, Tran T, et al. Pseudoaneurysm of the peroneal artery: presentation of Ehlers-Danlos syndrome type IV. Eur J Vasc Endovasc Surg. 2008;36:353–355.
6. Ascione R, Gomes WJ, Bates M, et al. Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome. Cardiovasc Surg. 2000;8:75–78.
7. Azghari A, Bouayad M, Belmir H, et al. Vascular type of Ehlers-Danlos syndrome: a rare cause of spontaneous pseudo-aneurysms [in French]. J Mal Vasc. 2010;35:254–258.
8. Badawi RA, Brent LH, Feinstein DE. Mimics of vasculitis: vascular Ehlers-Danlos syndrome masquerading as polyarteritis nodosa. J Rheumatol. 2009;36:1845–1847.
9. Bade MA, Queral LA, Mukherjee D, et al. Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. J Vasc Surg. 2007;46:360–362.
10. Barboi A, Dennis C, Timins M, et al. Neuromuscular manifestations in a patient with Ehlers-Danlos syndrome type IV. J Clin Neuromuscul Dis. 2009;11:81–87.
11. Bashir Q, Thornton J, Alp S, et al. Carotid-cavernous fistula associated with Ehlers-Danlos syndrome type IV. A case report and review of literature. Interv Neuroradiol. 1999;5:313–320.
12. Behjati S, Knight Y, Borgstein R, et al. A life-threatening complication of Ehlers-Danlos syndrome. Br J Hosp Med (Lond). 2009;70:360.
13. Benchellal ZA, Huten N, Danquechin Dorval E, et al. Abdominal emergencies in type IV Ehlers-Danlos syndrome [in French]. Gastroenterol Clin Biol. 1998;22:343–345.
14. Bjorck M, Pigg M, Kragsterman B, et al. Fatal bleeding following delivery: a manifestation of the vascular type of Ehlers-Danlos' syndrome. Gynecol Obstet Invest. 2007;63:173–175.
15. Bloch R, Hoffer E, Borsa J, et al. Ehlers-Danlos syndrome mimicking mesenteric vasculitis: therapy, then diagnosis. J Vasc Interv Radiol. 2001;12:527–529.
16. Borck G, Beighton P, Wilhelm C, et al. Arterial rupture in classic Ehlers-Danlos syndrome with COL5A1 mutation. Am J Med Genet A. 2010;152A:2090–2093.
17. Brees CK, Gall SA. Rupture of the external iliac artery during pregnancy: a case of type IV Ehlers-Danlos syndrome. J Ky Med Assoc. 1995;93:553–555.
18. Byard RW, Keeley FW, Smith CR. Type IV Ehlers-Danlos syndrome presenting as sudden infant death. Am J Clin Pathol. 1990;93:579–582.
19. Calvo P, Lanciego C, Krasniqi G, et al. Successful endovascular treatment of a splenic artery aneurysm in a patient with Ehlers-Danlos syndrome. J Vasc Interv Radiol. 2009;20:274–275.
20. Casana R, Nano G, Dalainas I, et al. Endovascular treatment of hepatic artery aneurysm in a patient with Ehlers-Danlos syndrome. Case report. Int Angiol. 2004;23:291–295.
21. Charlier P, Germain DP, Jeunemaitre X, et al. Sudden death associated to vascular Ehlers-Danlos syndrome. A case report. Leg Med (Tokyo). 2011;13:145–147.
22. Chuman H, Trobe JD, Petty EM, et al. Spontaneous direct carotid-cavernous fistula in Ehlers-Danlos syndrome type IV: two case reports and a review of the literature. J Neuroophthalmol. 2002;22:75–81.
23. Cikrit DF, Glover JR, Dalsing MC, et al. The Ehlers-Danlos specter revisited. Vasc Endovascular Surg. 2002;36:213–217.
24. Collins MH, Schwarze U, Carpentieri DF, et al. Multiple vascular and bowel ruptures in an adolescent male with sporadic Ehlers-Danlos syndrome type IV. Pediatr Dev Pathol. 1999;2:86–93.
25. Curley SA, Osler T, Demarest GB. Traumatic disruption of the subclavian artery and brachial plexus in a patient with Ehlers-Danlos syndrome. Ann Emerg Med. 1988;17:850–852.
26. Debrun GM, Aletich VA, Miller NR, et al. Three cases of spontaneous direct carotid cavernous fistulas associated with Ehlers-Danlos syndrome type IV. Surg Neurol. 1996;46:247–252.
27. Desal HA, Toulgoat F, Raoul S, et al. Ehlers-Danlos syndrome type IV and recurrent carotid-cavernous fistula: review of the literature, endovascular approach, technique and difficulties. Neuroradiology. 2005;47:300–304.
28. D'Souza CR, Hebert RJ, Trautman AF, et al. Aortoenteric fistula: case review and a new surgical technique. Can J Surg. 1987;30:415–417.
29. de Wazieres B, Coppere B, Durieu I, et al. Vascular and/or cardiac manifestations of type IV Ehlers-Danlos syndrome. 9 cases [in French]. Presse Med. 1995;24:1381–1385.
30. Eskandar O, Eckford SD. Pelvic haematoma and severe gluteal bruising associated with Ehler-Danlos syndrome following posterior colpoperineorrhaphy. J Obstet Gynaecol. 2009;29:68–69.
31. Freeman RK, Swegle J, Sise MJ. The surgical complications of Ehlers-Danlos syndrome. Am Surg. 1996;62(10):869–873.
32. Geisbusch P, Kotelis D, von Tengg-Kobligk H, et al. Thoracic aortic endografting in patients with connective tissue diseases. J Endovasc Ther. 2008;15:144–149.
33. Gelbmann CM, Kollinger M, Gmeinwieser J, et al. Spontaneous rupture of liver in a patient with Ehlers Danlos disease type IV. Dig Dis Sci. 1997;42:1724–1730.
34. Gilchrist ER, Duflou JA. Incidental myocardial infarction in Ehlers-Danlos syndrome type IV? J Forensic Sci. 2005;50:461–464.
35. Guyot E, Carillion A, Poli-Merol ML, et al. Ehlers-Danlos syndrome type IV in a child admitted in emergency with peritonitis. Br J Anaesth. 2009;103:615–616.
36. Hagspiel KD, Bonatti H, Sabri S, et al. Metachronous bilateral posterior tibial artery aneurysms in Ehlers-Danlos syndrome type IV. Cardiovasc Intervent Radiol. 2010;34:413–418.
37. Hamel BC, Pals G, Engels CH, et al. Ehlers-Danlos syndrome and type III collagen abnormalities: a variable clinical spectrum. Clin Genet. 1998;53:440–446.
38. Hansch A, Gajda M, Kaiser WA. Spontaneous rupture of the abdominal aorta in Ehlers-Danlos syndrome [in German]. Rofo. 2008;180:755–756.
39. Hollands JK, Santarius T, Kirkpatrick PJ, et al. Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome: a novel approach. Neuroradiology. 2006;48:491–494.
40. Horowitz MB, Purdy PD, Valentine RJ, et al. Remote vascular catastrophes after neurovascular interventional therapy for type 4 Ehlers-Danlos Syndrome. AJNR Am J Neuroradiol. 2000;21:974–976.
41. Hosaka A, Miyata T, Shigematsu H, et al. Spontaneous mesenteric hemorrhage associated with Ehlers-Danlos syndrome. J Gastrointest Surg. 2006;10:583–585.
42. Hovsepian DM, Aguilar RL, Sicard GA, et al. Stent-graft failure in a patient with a connective tissue disorder. J Vasc Interv Radiol. 1997;8:789–793.
43. Iida Y, Obitsu Y, Komai H, et al. Successful coil embolization for rupture of the subclavian artery associated with Ehlers-Danlos syndrome type IV. J Vasc Surg. 2009;50:1191–1195.
44. Im JS, Lim YH, Park JS, et al. Rupture of abdominal aortic aneurysm after spine surgery in the patient with Ehlers-Danlos syndrome: a case report. Korean J Anesthesiol. 2010;58:555–559.
45. Imamura A, Nakamoto H, Inoue T, et al. Ruptured dissecting aneurysm in bilateral iliac arteries caused by Ehlers-Danlos syndrome type IV: report of a case. Surg Today. 2001;31:85–89.
46. Jarmulowicz M, Phillips WG. Vascular Ehlers-Danlos syndrome undiagnosed during life. J R Soc Med. 2001;94:28–30.
47. Kanner AA, Maimon S, Rappaport ZH. Treatment of spontaneous carotid-cavernous fistula in Ehlers-Danlos syndrome by transvenous occlusion with Guglielmi detachable coils. Case report and review of the literature. J Neurosurg. 2000;93:689–692.
48. Karkos CD, Prasad V, Mukhopadhyay U, et al. Rupture of the abdominal aorta in patients with Ehlers-Danlos syndrome. Ann Vasc Surg. 2000;14:274–277.
49. Kato T, Hattori H, Yorifuji T, et al. Intracranial aneurysms in Ehlers-Danlos syndrome type IV in early childhood. Pediatr Neurol. 2001;25:336–339.
50. Khalique Z, Lyons OT, Clough RE, et al. Successful endovascular repair of acute type B aortic dissection in undiagnosed Ehlers-Danlos syndrome type IV. Eur J Vasc Endovasc Surg. 2009;38:608–609.
51. Koh JH, Kim JS, Hong SC, et al. Skin manifestations, multiple aneurysms, and carotid-cavernous fistula in Ehlers-Danlos syndrome type IV. Circulation. 1999;100:e57–e58.
52. Kurata A, Oka H, Ohmomo T, et al. Successful stent placement for cervical artery dissection associated with the Ehlers-Danlos syndrome. Case report and review of the literature. J Neurosurg. 2003;99:1077–1081.
53. Lauwers G, Nevelsteen A, Daenen G, et al. Ehlers-Danlos syndrome type IV: a heterogeneous disease. Ann Vasc Surg. 1997;11:178–182.
54. Levine MP. Survival of an aortic trauma patient with Ehlers-Danlos syndrome type IV: a case report. J Vasc Surg. 2000;32:1219–1221.
55. Lim SP, Duddy MJ. Endovascular treatment of a carotid dissecting pseudoaneurysm in a patient with Ehlers-Danlos syndrome type IV with fatal outcome. Cardiovasc Intervent Radiol. 2008;31:201–204.
56. Lipinski MJ, Lipinski SE, Kripalani S, et al. An unusual presentation of Ehlers-Danlos syndrome vascular type with deep vein thrombosis: a case for multidisciplinary management. Am J Med Genet A. 2009;149A:698–701.
57. Maltz SB, Fantus RJ, Mellett MM, et al. Surgical complications of Ehlers-Danlos syndrome type IV: case report and review of the literature. J Trauma. 2001;51:387–390.
58. Matsushima K, Takara H. Endovascular treatment for a spontaneous rupture of the posterior tibial artery in a patient with Ehlers-Danlos syndrome type IV: report of a case. Surg Today. 2009;39:523–526.
59. Meldon S, Brady W, Young JS. Presentation of Ehlers-Danlos syndrome: iliac artery pseudoaneurysm rupture. Ann Emerg Med. 1996;28:231–234.
60. Mitsuhashi T, Miyajima M, Saitoh R, et al. Spontaneous carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV—case report. Neurol Med Chir (Tokyo). 2004;44:548–553.
61. Musumeci A, Minervini MI, Cintorino D, et al. Postoperative hepatic artery aneurysms development and remodeling in Ehlers-Danlos syndrome type IV. Case report. Int Angiol. 2008;27:166–169.
62. Naidu SG, Chong BW, Huettl EA, et al. Percutaneous embolization of a lumbar pseudoaneurysm in a patient with type IV Ehlers-Danlos syndrome. J Vasc Surg. 2007;46:1036–1038.
63. Nakamura M, Yajima J, Oikawa Y, et al. Vascular Ehlers-Danlos syndrome—all three coronary artery spontaneous dissections. J Cardiol. 2009;53:458–462.
64. Nanayakkara PW, van Bunderen CC, Simsek S, et al. Vascular type of Ehlers-Danlos syndrome in a patient with ruptured aneurysm of the splenic artery. Neth J Med. 2006;64:374–376.
65. Nosher JL, Trooskin SZ, Amorosa JK. Occlusion of a hepatic arterial aneurysm with gianturco coils in a patient with the Ehlers-Danlos syndrome. Am J Surg. 1986;152:326–328.
66. Olgunturk FR, Tunaoglu FS, Gumus H, et al. Diffuse arterial aneurysms in a case of Ehlers-Danlos syndrome—a case report. Angiology. 1993;44:909–913.
67. Parfitt J, Chalmers RT, Wolfe JH. Visceral aneurysms in Ehlers-Danlos syndrome: case report and review of the literature. J Vasc Surg. 2000;31:1248–12451.
68. Pickup MJ, Pollanen MS. Traumatic subarachnoid hemorrhage and the COL3A1 gene: emergence of a potential causal link. Forensic Sci Med Pathol. 2010;7:192–197.
69. Prahlow JA, Wagner SA. Death due to Ehlers-Danlos syndrome type IV. Am J Forensic Med Pathol. 2005;26:78–82.
70. Privitera A, Milkhu C, Datta V, et al. Spontaneous rupture of the spleen in type IV Ehlers-Danlos syndrome: report of a case. Surg Today. 2009;39:52–54.
71. Rossi PI, Scher LA, Friedman SG, et al. Subclavian artery pseudoaneurysm in type IV Ehlers-Danlos syndrome. J Vasc Surg. 1998;27:549–551.
72. Schievink WI. Cerebrovascular involvement in Ehlers-Danlos syndrome. Curr Treat Options Cardiovasc Med. 2004;6:231–236.
73. Schievink WI, Limburg M, Oorthuys JW, et al. Cerebrovascular disease in Ehlers-Danlos syndrome type IV. Stroke. 1990;21:626–632.
74. Sherry S, Fletcher A, Alkjaersig N, et al. An approach to intravascular fibrinolysis in man. Trans Assoc Am Physicians. 1957;70:288–296.
75. Shields LB, Rolf CM, Davis GJ, et al. Sudden and unexpected death in three cases of Ehlers-Danlos syndrome type IV. J Forensic Sci. 2010;55:1641–1645.
76. Snyder RR, Gilstrap LC, Hauth JC. Ehlers-Danlos syndrome and pregnancy. Obstet Gynecol. 1983;61:649–650.
77. Sugawara Y, Ban K, Imai K, et al. Successful coil embolization for spontaneous arterial rupture in association with Ehlers-Danlos syndrome type IV: report of a case. Surg Today. 2004;34:94–96.
78. Sultan S, Morasch M, Colgan MP, et al. Operative and endovascular management of extracranial vertebral artery aneurysm in Ehlers-Danlos syndrome: a clinical dilemma–case report and literature review. Vasc Endovascular Surg. 2002;36:389–392.
79. Tonnessen BH, Sternbergh WC III, Mannava K, et al. Endovascular repair of an iliac artery aneurysm in a patient with Ehlers-Danlos syndrome type IV. J Vasc Surg. 2007;45:177–179.
80. Whinney D, Nicholson S, Ridley P. Surgical presentations of Ehlers-Danlos syndrome type IV. A case report. J Cardiovasc Surg (Torino). 1994;35:559–560.
81. Wimmer PJ, Howes DS, Rumoro DP, et al. Fatal vascular catastrophe in Ehlers-Danlos syndrome: a case report and review. J Emerg Med. 1996;14:25–31.
82. Witz M, Lehmann JM. Aneurysmal arterial disease in a patient with Ehlers-Danlos syndrome. Case report and literature review. J Cardiovasc Surg (Torino). 1997;38:161–163.
83. Vogel LC, Lubicky JP. Neurologic and vascular complications of scoliosis surgery in patients with Ehlers-Danlos syndrome. A case report. Spine (Phila Pa 1976). 1996;21:2508–2514.
84. Yang JS, Sponseller PD, Yazici M, et al. Vascular complications from anterior spine surgery in three patients with Ehlers-Danlos syndrome. Spine (Phila Pa 1976). 2009;34:E153–E157.
85. Yilmaz A, Dessing T, Botta L. Ascending aorta impending rupture: successful surgical management in a patient with type IV Ehlers-Danlos syndrome. J Cardiovasc Med (Hagerstown). 2010;11:610–612.
86. Yost BA, Vogelsang JP, Lie JT. Fatal hemoptysis in Ehlers-Danlos syndrome. Old malady with a new curse. Chest. 1995;107:1465–1467.
87. Hamano K, Minami Y, Fujimura Y, et al. Emergency operation for thoracic aortic aneurysm caused by the Ehlers-Danlos syndrome. Ann Thorac Surg. 1994;58:1180–1182.
88. Evans RH, Fraser AG. Spontaneous coronary artery rupture and cardiac tamponade in Ehlers-Danlos syndrome type IV. Int J Cardiol. 1996;54:283–286.
89. Athanassiou AM, Turrentine MA. Myocardial infarction and coronary artery dissection during pregnancy associated with type IV Ehlers-Danlos syndrome. Am J Perinatol. 1996;13:181–183.
90. Oderich GS, Panneton JM, Bower TC, et al. The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: a 30-year experience. J Vasc Surg. 2005;42:98–106.
91. Brooke BS. Celiprolol therapy for vascular Ehlers-Danlos syndrome. Lancet. 2010;376:1443–1444.
92. Shimaoka Y, Kosho T, Wataya-Kaneda M, et al. Clinical and genetic features of 20 Japanese patients with vascular-type Ehlers-Danlos syndrome. Br J Dermatol. 2010;163:704–710.
93. Swedvasc. Annual Report. 2010. www.karlkirurgi.com. Accessed June 4, 2013.
94. Mast KJ, Nunes ME, Ruymann FB, et al. Desmopressin responsiveness in children with Ehlers-Danlos syndrome associated bleeding symptoms. Br J Haematol. 2009;144:230–233.
95. Ong KT, Perdu J, De Backer J, et al. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet. 2010;376:1476–1484.
96. Van Hemelrijk C, Renard M, Loeys B. The Loeys-Dietz syndrome: an update for the clinician. Curr Opin Cardiol. 2010;25:546–551