1/67. Multiple aortocaval fistulas associated with a ruptured abdominal aneurysm in a patient with ehlers-danlos syndrome.Aortocaval fistula (ACF) is a rare complication of spontaneous abdominal aortic aneurysm (AAA) rupture, with an incidence of 2-4%. A unique case of ruptured AAA complicated by multiple aortovenous fistulas involving the inferior vena cava and left internal iliac vein is presented, and is the first published report of a patient with ehlers-danlos syndrome undergoing surgical treatment for an ACF.- - - - - - - - - - ranking = 1keywords = aneurysm (Clic here for more details about this article) |
2/67. Remote vascular catastrophes after neurovascular interventional therapy for type 4 ehlers-danlos syndrome.Type 4 ehlers-danlos syndrome (EDS 4) is the most malignant form of ehlers-danlos syndrome, often accompanied by neurovasacular complications secondary to vessel dissection or aneurysms. The fragile nature of connective tissue in these patients makes exovascular and endovascular treatment hazardous. We have treated four patients with EDS 4 over the last 8 years by using neuroendovascular procedures. Two of these individuals suffered remote vascular injuries around the time of their procedures and ultimately died. The circumstances surrounding their deaths will make up the body of this report.- - - - - - - - - - ranking = 0.2keywords = aneurysm (Clic here for more details about this article) |
3/67. Visceral aneurysms in ehlers-danlos syndrome: case report and review of the literature.A patient with ehlers-danlos syndrome type IV had a celiac artery aneurysm. There are only nine previously published cases of visceral artery aneurysms in this condition, and surgical management was used in only four of them. This is, we believe, the first published report of a successfully treated celiac artery aneurysm in this condition in which the hazards of treatment and our rationale are explained. The literature of this rare problem is also reviewed.- - - - - - - - - - ranking = 1.4keywords = aneurysm (Clic here for more details about this article) |
4/67. Ruptured dissecting aneurysm in bilateral iliac arteries caused by Ehlers-Danlos syndrome type IV: report of a case.ehlers-danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and abnormalities of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized based on genetic, clinical, and biochemical characteristics. The spectrum of the disorder varies from mild to life-threatening vascular complications. EDS type IV is a particularly dangerous form with a lethal spontaneous rupture of the major arteries and aneurysmal formation. We present herein a case of a ruptured dissecting aneurysm in the bilateral iliac arteries caused by EDS type IV. A previously healthy 33-year-old man without any physical features of this connective tissue disorder experienced a metachronous vascular rupture two times. Successful synthetic bypass grafting was performed with great difficulty. The diagnosis of EDS type IV was made afterwards based on an electrophoresis analysis of a skin biopsy specimen which revealed a lack of type III collagen. Surgical intervention in cases of arterial complications in EDS type IV patients have been reported to be both difficult and frequently unsuccessful. The early clinical recognition of this syndrome is therefore of great importance due to the hazards of such surgical therapies.- - - - - - - - - - ranking = 1.2keywords = aneurysm (Clic here for more details about this article) |
5/67. ehlers-danlos syndrome type IV and multiple aortic aneurysms--a case report.Beside atherosclerosis, aortic aneurysms can be part of the clinical spectrum of many systemic diseases, including infectious, inflammatory, genetic and, less often, congenital disorders. A 48-year-old white man presented with multiple large aneurysms of the aorta and its main branches. Medical history was unremarkable except for the presence of a softened abdominal mass since he was 28 years old. On the physical examination, an arterial murmur was heard over the left carotid artery and a palpable mass was noted in the whole right side of the abdomen. No skin or joint abnormalities were noted. aortography, computed tomography, and magnetic resonance angiography showed multiple large aneurysms of the descending thoracic and abdominal aorta. Aneurysms of the innominate, left subclavian, and carotid arteries were also seen. This case resembles those previously reported, in which multiple aortic aneurysms were associated with abnormalities of the type III procollagen gene (COL3A1). Although the classic stigmas of the ehlers-danlos syndrome type IV were lacking, this genetic disease may be the cause of the multiple aneurysms in this patient.- - - - - - - - - - ranking = 1.8keywords = aneurysm (Clic here for more details about this article) |
6/67. A brachial aneurysm in childhood caused by ehlers-danlos syndrome.A case of an 11-year-old boy with ehlers-danlos syndrome (EDS) type IV is presented. He was referred with a pulsatile mass above the right antecubital fossa and Doppler revealed a saccular aneurysm. Excision and ligation of the brachial artery aneurysm was performed. The diagnosis was confirmed by skin fibroblast cultures and histopathologic evaluation. We conclude that arteriography should be avoided in EDS and color-Doppler should be the sole means of diagnosis. ligation rather than arterial reconstruction should be preferred.- - - - - - - - - - ranking = 1.2keywords = aneurysm (Clic here for more details about this article) |
7/67. Intracranial aneurysms in ehlers-danlos syndrome type IV in early childhood.ehlers-danlos syndrome type IV is of special interest to neurologists because of the risk of cerebrovascular complications. We describe a 5-year-old female with ehlers-danlos syndrome type IV, demonstrating multiple intracranial aneurysms and right middle cerebral artery stenosis. The diagnosis of ehlers-danlos syndrome type IV was confirmed by electron microscopic examination of a skin biopsy. To our knowledge, this is the youngest reported patient with intracranial aneurysms associated with the ehlers-danlos syndrome type IV. ehlers-danlos syndrome type IV should be considered in the differential diagnosis of cerebrovascular disorder and stroke in early childhood.- - - - - - - - - - ranking = 1.2keywords = aneurysm (Clic here for more details about this article) |
8/67. The Ehlers-Danlos specter revisited.Ehlers-Danlos type IV is a major concern to vascular surgeons because it is often associated with spontaneous hemorrhage from arteries containing decreased type III collagen. Five members of a family with Ehlers-Danlos type IV and a review of another family of five with Ehlers-Danlos type IV are reported. Evaluation of the recent family included clinical evaluation as well as assay of collagen production. The age range of the three involved females and two males was 7 to 52 years. The father of the affected family had a spontaneous colon perforation at age 39. His son, at age 27, had a spontaneous rupture of the iliac artery. Revascularization was accomplished with difficulty. His daughter had a large cerebral bleed. Two granddaughters, ages 7, have not had any bleeding or aneurysmal events. The amount of type III collagen was only 10% of normal in the patient with the iliac artery rupture. The three females all exhibited similarly low levels of type III collagen. The father's type III collagen level was not sufficiently low to confirm Ehlers-Danlos type IV, although he had a spontaneous colon perforation. In the other Ehlers-Danlos type IV family of five, the three surviving members had type III collagen levels as low as 5% of normal. Two family members died after spontaneous iliac rupture at ages 24 and 33. Both families exhibited an autosomal dominant inheritance pattern. Ehlers-Danlos type IV remains a challenging problem for vascular surgeons. It is transmitted as an autosomal dominant inheritance with a high degree of penetrance. Spontaneous arterial and intestinal perforations should alert the clinician to the possibility of Ehlers-Danlos type IV. patients should be evaluated noninvasively. Arterial repairs may not be successful in these patients because the vessels are extremely friable. Assays of collagen production are advisable in establishing the diagnosis.- - - - - - - - - - ranking = 0.2keywords = aneurysm (Clic here for more details about this article) |
9/67. ehlers-danlos syndrome type IV and a novel mutation of the type III procollagen gene as a cause of abdominal apoplexy.Abdominal apoplexy is a clinical entity characterized by spontaneous intraperitoneal hemorrhage from rupture of a visceral vessel. We describe a 34-year-old man who presented with abdominal apoplexy due to rupture of an ileocolic aneurysm. Subsequent biochemical and genetic analysis confirmed the diagnosis of ehlers-danlos syndrome type IV based on abnormal production of type III procollagen and a novel mutation in the COL3A1 gene. patients presenting with abdominal apoplexy should undergo a thorough examination so that the underlying vascular pathology can be identified.- - - - - - - - - - ranking = 0.2keywords = aneurysm (Clic here for more details about this article) |
10/67. intracranial aneurysm surgery in ehlers-danlos syndrome Type IV.OBJECTIVE: ehlers-danlos syndrome Type IV is a heritable connective tissue disorder with frequent neurovascular manifestations, such as intracranial aneurysms. patients with this syndrome have notoriously fragile blood vessels, and the reported mortality rate for any type of vascular surgical procedure is 40%. This syndrome is rare, however, and the complication rate of aneurysm surgery may have been overestimated. methods: We reviewed our experience with aneurysm surgery in a group of patients with ehlers-danlos syndrome Type IV. RESULTS: The patient population consisted of three women and one man with a mean age of 44 years (age range, 20-57 yr). One patient, who had a ruptured anterior circulation aneurysm, died as a direct result of surgery because of marked vascular fragility. Three patients underwent successful surgery, consisting of a craniotomy and clipping of a ruptured anterior circulation aneurysm in two patients and a craniotomy and clip ligation of the parent artery in one patient with a ruptured dissecting vertebral artery aneurysm. Intraoperatively, mild vascular or connective tissue fragility was commonly observed. postoperative complications (e.g., spontaneous pneumothorax and vertebral artery dissection) also were common but did not result in permanent morbidity. CONCLUSION: The risk of neurovascular surgery is high in patients with ehlers-danlos syndrome Type IV and intra- and postoperative complications are common. However, most patients tolerate the operation without permanent morbidity.- - - - - - - - - - ranking = 2keywords = aneurysm (Clic here for more details about this article) |
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