Ehlers—Danlos syndrome type IV is a distinctive syndrome in which thin and fragile skin, premature ageing, bruising and scarring are combined with lethal or life-threatening arterial weakness Aortic rupture either at the aortic root and arch, or sometimes lower down the artery, are Darticularly characteristic. Even quite minor injury can produce dangerous vascular tearing and damage Technical difficulties encountered in arterial repair or venous ligature are particularly worrvinq The authors report the treatment of a ruptured type A aortic dissection associated with Ehlers—Danlos syndrome where the extreme fragility of the tissues and tendency to bleed posed a difficult task for the surgeon.
De PaepeA., The Ehlers—Danlos syndrome: A heritable collagen disorder as cause of bleeding. Thrombosis and Haemostasis, 1996, 75, 379–386.
2.
PopeF. M.NichollsA. C.NarcisiP., Type III collagen mutations in Ehlers—Danlos syndrome, type IV and other related disorders. Clinical and Experimental Dermatology, 1988, 13, 285–302.
3.
SteinmannB.RoyceP.M.Superti-FurgaA., The Ehlers—Danlos syndrome. In Connective Tissue and its Heritable Disorders. Molecular, Genetic and Medical Aspects, ed. RoyceP. M.SteinmannB.. Wiley—Liss, New York, 1993, pp. 351–407.
4.
BergqvistD., Ehlers—Danlos type IV syndrome. A review from a vascular surgical point of view. European Journal of Surgery, 1996, 162, 163–170.
5.
SerryC.AgomuohO. S.GoldinM. D., Review of Ehlers—Danlos syndrome: Successful repair of rupture and dissection of abdominal aorta. Journal of Cardiovascular Surgery, 1988, 29, 530–534.
6.
RamanJ.SaldanhaR. F.EsmoreD. S., The Bentall procedure: A surgical option in Ehlers—Danlos syndrome. Journal of Cardiovascular Surgery, 1988, 29, 647–649.
7.
BrearleyS.FowlerJ.HamerJ. D., Two vascular complications of the Ehlers—Danlos syndrome. European Journal of Vascular Surgery, 1993, 7, 210–213.
8.
MattarS. G.KumarA. G.LumsdenA. B., Vascular complications in Ehlers—Danlos syndrome. American Surgery, 1994, 60, 827–831.
