1/35. 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 = 1keywords = vessel (Clic here for more details about this article) |
2/35. survival of an aortic trauma patient with ehlers-danlos syndrome type IV: a case report.ehlers-danlos syndrome type IV is the most lethal variant of that illness and is associated with fatal large vessel arterial hemorrhages. The literature reports only two survivors of elective aortic surgery and two survivors of spontaneous aortic hemorrhage. This article presents a 14-year-old boy who had aortic and vena cava blunt trauma and survived.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/35. Long-term survival in truncus arteriosus communis type A1 associated with ehlers-danlos syndrome--a case report.Long-term survival is rare in patients with truncus arteriosus communis type A1 due to the early appearance of pulmonary artery obstructive changes. A 48-year-old woman with truncus arteriosus communis type A1 diagnosed with chest radiography, two-dimensional echocardiography, and cardiac catheterization is presented. The right ventricular pressure was 108 mm Hg, whereas the systolic pulmonary artery pressure was 98 mm Hg. A concomitant diagnosis of the ehlers-danlos syndrome was made with clinical findings. The association of ehlers-danlos syndrome, in which a collagen synthesis defect is observed in arterial vessels, might have interfered with pulmonary artery intimal proliferation, thus precluding the appearance of advanced pulmonary artery luminal obstructive changes and allowing long-term survival.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/35. 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 = 1keywords = vessel (Clic here for more details about this article) |
5/35. 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 = 1keywords = vessel (Clic here for more details about this article) |
6/35. 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 = 19.192971379838keywords = blood vessel, vessel (Clic here for more details about this article) |
7/35. Post traumatic subconjunctival dislocation of lens in ehlers-danlos syndrome.Ehlers-Danlos Syndromes are a rare group of inherited connective tissue disorders of defective collagen synthesis. They predominantly involve the skin, joints and vessels. Ocular involvement in the form of blue sclera, microcornea and susceptibility to trauma is a common feature of EDS type VI. A minor ocular trauma in these patients can cause globe rupture. We herein report simple and successful management of a case of traumatic subconjunctival dislocation of lens in a patient of EDS type VI.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/35. Safe ECT in a patient with the ehlers-danlos syndrome.The ehlers-danlos syndrome is an inheritable disorder caused by genetic defects of collagen. Hyperextensibility and fragility of the skin, joint hyperlaxity, and generalized fragility of the connective tissues, involving the hollow organs and arteries, are the cardinal features of this syndrome. Low bone density has also been reported. Theoretically, these patients can have an increased risk of rupture of blood vessels and of bone fractures. A patient with a schizo-affective disorder and Ehlers-Danlos Type I who received bifrontal ECT for a depressive episode is described. Even without specific measures to lower blood pressure, a course of ECT was finished without any complication.- - - - - - - - - - ranking = 19.192971379838keywords = blood vessel, vessel (Clic here for more details about this article) |
9/35. Coronary arteriomegaly in a patient with ehlers-danlos syndrome and multiple aneurysms--a case report.The authors report a case of coronary arteriomegaly in a patient with ehlers-danlos syndrome and multiple aneurysms who presented with myocardial infarction. Coronary arteriography revealed distal "pruning" of vessels without occlusive disease. Fibroblast cultures excluded ehlers-danlos syndrome types IV and VII. literature review suggests this may represent a previously unidentified vascular syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/35. Microangiopathy in ehlers-danlos syndrome type IV.In two patients with ehlers-danlos syndrome type IV, an autosomal dominant disorder characterized by fragility of large vessels, excessive bruising, and deficiency in type III collagen, capillary microscopy was performed at the nailfold. indocyanine green and Na-fluorescein were used as fluorescent tracers. Both patients exhibited microangiopathy of the skin capillaries with microbleedings, presence of microaneurysms and increased transcapillary diffusion. Microvascular involvement appears to be an additional manifestation of the syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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