Spontaneous arterial perforation: The Ehlers-Danlos specter

Abstract

The Ehlers-Danlos syndrome (EDs) is one of the most frequently inherited disorders of connective tissue. Type IV EDs, the arterial-ecchymotic type, is of concern to vascular surgeons because it is frequently associated with spontaneous catastrophic bleeding. This article summarizes our experience with five members of a family and reviews the 31 patients with type IV EDs described in the literature. The 22 male and 14 female patients had a mean age of 26 years. The 36 patients included 23 with easy "bruisability," 22 with hypermobility of their joints (especially the fingers), 13 with transparent skin, and 11 with excessive elasticity of the skin. The patients had 41 episodes of hemorrhage and 29 vascular surgical procedures. Twenty-nine of the patients had an aneurysm or a dissection, whereas eight patients had arteriovenous fistulas. Arteriography was associated with a complication rate of 67%. The prognosis for a patient with type IV EDs is poor; 44% die before surgery and 19% die during the operative period. Bleeding should be managed nonoperatively when possible. Fifty-one percent die before reaching 40 years of age. Arteriography should be avoided. The standard repair of aneurysms and perforations is unlikely to be successful because of marked vessel friability. Bleeding vessels should be repaired with buttressed sutures and no tension or should be ligated. Genetic counseling with assay of collagen production is recommended for family members.

Spontaneous Carotid-Cavernous Fistula in a Patient With Ehlers-Danlos Syndrome Type IV-Case Report-

“…4,10,23,31) In this case, it was judged that the risk of performing intravascular catheter operation by the fragile of a vessel wall was very high. We gave up performing some inspection of monitoring before operation, such as cerebral blood flow study with balloon occlusion examination, and so on.…”

Clinical Reasoning: A 21-year-old woman with right eye swelling and bruising

“…9 In the general population, angiography carries a risk of 2.5% morbidity, but in patients with EhlersDanlos type IV, angiography has been associated with morbidity rates of 36%-67% because of the increased risk of carotid dissection, arterial perforation, and hematoma. 4,10 Both the transarterial and transvenous approaches carry these high risks because of the fragility of the vessels. Arteries in these patients are also often more tortuous, making it difficult to navigate a catheter through the vasculature.…”

Percutaneous Interventions in Aortic Disease

“…One case series examining 31 family members with Ehlers-Danlos aortic disease revealed that 8 of 12 patients who underwent percutaneous diagnostic or interventional angiographic procedures had access site complications, including 2 deaths. 64 Other literature supports open femoral artery exposure for any endovascular procedure to primarily repair the diseased artery in a tension-free fashion, often with Teflon felt pledgets or cuffs. 65,66 With the high number and frequency of vascular complications in these patients during open femoral exposure for endovascular access, it is not surprising that no one has been eager to attempt percutaneous aortic interventions.…”