Cases reported "Ehlers-Danlos Syndrome"

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1/22. ehlers-danlos syndrome: surgical management of mitral regurgitation and atrial fibrillation.

    ehlers-danlos syndrome is associated with fatal cardiovascular complications and intraoperative death. The lack of literature on cardiac surgery in the syndrome makes it difficult for surgeons to assess the risk of an operation and to choose optimal treatment. We describe the case of a 63-year-old man with ehlers-danlos syndrome type I or II, mitral regurgitation and atrial fibrillation who was managed successfully with mitral valve repair and surgical cardioversion.
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keywords = vascular complication
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2/22. 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.
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keywords = vascular complication
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3/22. 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.
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ranking = 1
keywords = vascular complication
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4/22. Fatal cardiovascular complications in a patient with ehlers-danlos syndrome type IV and dextrocardia.

    A report is presented of a 31 year female patient with ehlers-danlos syndrome type IV who presented a bilateral renal artery dissection and a hematoma of the ascending aorta simultaneously. She had had an uneventful delivery six months before. We also discovered a situs solitus with dextrocardia in this patient. These complications were managed conservatively. Unfortunately the patient died of a pulmonary infection and a multi-organ failure syndrome several days after her admission.
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ranking = 4
keywords = vascular complication
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5/22. Surgical management of the spinal deformity in ehlers-danlos syndrome type VI.

    Five patients with ehlers-danlos syndrome type VI were surgically treated for their spinal deformities. All of them were female. All but one had a double-major thoracic and lumbar curve. One had a mild scoliotic curve but severe thoracic lordosis. Anterior discectomy and fusion and posterior instrumentation was performed in a single stage in two patients, while two had anterior and posterior surgery performed in two stages. The remaining patient underwent posterior surgery only, because of the relative mildness of the deformity and adequate flexibility. Two serious vascular complications were confronted. One patient experienced avulsion of segmental arteries and rupture of iliac artery and vein during anterior surgery. Another patient had avulsion of superior gluteal artery, which happened during subperiosteal dissection to harvest iliac bone graft. Fortunately, we did not see any temporary or permanent neurological complications. The spinal fusions appeared solid radiographically, with no implant failure and loss of correction after an average follow-up of 4 years.
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ranking = 1
keywords = vascular complication
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6/22. Successful coil embolization for spontaneous arterial rupture in association with ehlers-danlos syndrome type IV: report of a case.

    When a patient with ehlers-danlos syndrome (EDS) presents with a vascular emergency, performing life-saving surgery can be difficult because of the profound fragility of the arterial tissue. We report the case of a 27-year-old woman with EDS in whom a spontaneous arterial rupture was successfully treated with transcatheter embolization. The patient was brought to our hospital in shock, with left lower abdominal pain. She had been diagnosed with EDS type IV following a colonic rupture 8 years earlier. An emergency angiogram revealed rupture of the left external iliac artery. The active bleeding was managed by transarterial embolization of the ruptured artery using stainless steel coils, which took 30 min to achieve. The patient has not suffered any further vascular complications during the year since this procedure. Transcatheter coil embolization may be a reliable option for treating sudden arterial rupture in patients with this syndrome.
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ranking = 1
keywords = vascular complication
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7/22. Successful treatment with the Smith-Hodge pessary of cervical incompetence due to defective connective tissue in ehlers-danlos syndrome.

    ehlers-danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders with at least ten distinct types. We report a case of EDS complicated by cervical incompetence. Treatment was undertaken after confirmation that the patient's EDS was not type IV, hence not associated with potentially serious vascular complications. Because of concern about stretching or tearing of the hyperextensible connective tissue associated with EDS, cerclage was not performed. The cervical incompetence due to defective connective tissue was treated with a Smith-Hodge pessary.
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ranking = 1
keywords = vascular complication
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8/22. Cerebrovascular disease in ehlers-danlos syndrome type IV.

    We describe two patients with cerebrovascular complications of ehlers-danlos syndrome type IV. A 16-year-old girl with spontaneous internal carotid artery dissection and a 46-year-old woman with aneurysmal subarachnoid hemorrhage and multiple aortic dissections were both deficient in collagen type iii, analyzed in cultured skin fibroblasts. To our knowledge, spontaneous carotid artery dissection associated with collagen type iii deficiency has not been reported previously. Early clinical recognition of this syndrome is of great importance in view of the hazards of angiography and surgery. collagen type iii deficiency plays a role in the pathogenesis of intracranial saccular aneurysms and may also be involved in the pathogenesis of carotid cavernous fistulas and dissections of the cervical arteries.
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ranking = 1
keywords = vascular complication
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9/22. Internal carotid artery aneurysm: a vascular manifestation of type IV ehlers-danlos syndrome.

    Aneurysms of the distal internal carotid artery (ICA) can be difficult to manage. When seen in a patient with ehlers-danlos syndrome, type IV (EDS-Type IV), the complexity of the problem is greatly increased. Proximal ICA ligation in the presence of an intact circle of willis and adequate collateral circulation is a reasonable and safe approach. We recommend the liberal use of duplex scanning and intravenous digital angiography in patients with EDS, and plan to use these techniques to follow the small, asymptomatic, right ICA aneurysm in our patient. If the aneurysm rapidly grows or becomes symptomatic, treatment may require proximal ligation with extracranial-intracranial bypass. Understanding of the basic defects associated with EDS-Type IV will make the management of patients with vascular complications more safe.
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ranking = 1
keywords = vascular complication
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10/22. Cardiovascular complications in the ehlers-danlos syndrome with minimal external findings.

    ehlers-danlos syndrome (EDS) is clinically and genetically a heterogeneous disorder of connective tissue. Eleven different types of EDS have been documented, several of which have major cardiovascular complications as part of their clinical manifestations. The purpose of this report is to call attention to a form of EDS with minimal external features but severe internal vascular complications.
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ranking = 6
keywords = vascular complication
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