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1/60. De novo development of a cavernous malformation of the spinal cord following spinal axis radiation. Case report.

    Analysis of recent reports has suggested that cavernous malformations (CMs) of the brain may have an acquired pathogenesis and a dynamic pathophysiological composition, with documented appearance of new lesions in familial cases and following radiotherapy. The authors report the first case of demonstrated de novo formation of an intramedullary CM following spinal radiation therapy. A 17 year-old boy presented with diabetes insipidus and delayed puberty. Evaluation of endocrine levels revealed hypopituitarism, and magnetic resonance (MR) imaging demonstrated an infundibular mass. The patient underwent a pterional craniotomy and removal of an infundibular germinoma. The MR image of the spine demonstrated normal results. The patient received craniospinal radiation therapy and did well. He presented 5 years later with acute onset of back pain, lower-extremity weakness and numbness, and difficulty with urination. An MR image obtained of the spine revealed an intramedullary T-7 lesion; its signal characteristics were consistent with a CM. The patient was initially managed conservatively but developed progressive myelopathy and partial brown-sequard syndrome. Although he received high-dose steroids and bed rest, his symptoms worsened. He underwent a costotransversectomy and excision of a hemorrhagic vascular lesion via an anterolateral myelotomy. Pathological examination confirmed features of a CM. The patient has done well and was walking without assistance within 4 weeks of surgery. De novo genesis of CMs may be associated with prior radiation therapy to the spinal cord.
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2/60. Subdural hematoma from a Type I spinal arteriovenous malformation. Case report.

    The authors report a patient in whom a subdural hematoma developed from a Type I spinal arteriovenous malformation (AVM). The patient became symptomatic with back pain, and magnetic resonance imaging revealed a spinal subdural hematoma. Selective spinal angiography, however, failed to demonstrate a pathological process. The patient underwent exploratory laminoplasty that revealed a subdural extraarachnoid hematoma with an underlying Type I spinal AVM, which was surgically obliterated. The patient recovered completely. Subdural hematomas that affect the spine are rare. Although a negative result was obtained using selective spinal angiography, exploratory surgery should be considered for the evacuation of a subdural hematoma and possibly for the definitive treatment of a spinal AVM.
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3/60. A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease.

    INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. methods: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.
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keywords = muscle
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4/60. Multiple gigantic arteriovenous malformations with destruction of lumbar vertebral bodies. A case report.

    STUDY DESIGN: This case report describes a patient with massive intramuscular and paravertebral arteriovenous malformations with destruction of vertebral bodies. OBJECTIVES: To demonstrate successful interbody fusion of the involved vertebral bodies after embolization for arteriovenous malformation. SUMMARY OF BACKGROUND DATA: Although arteriovenous malformations in the spinal cord are well documented in the literature, arteriovenous malformation in the paravertebral and iliopsoas muscles with destruction of vertebral bodies is an extremely rare clinical condition. methods: After careful investigation with angiography, the arteriovenous malformations were managed with embolization, and the scoliosis caused by the collapsed vertebral bodies was managed surgically by anterior spinal fusion with segmental spinal instrumentation. RESULTS: The patient's scoliosis caused by vertebral collapse was corrected by surgery, and good alignment of the lumbar spine was achieved. The preoperation pain had subsided completely by follow-up assessment 1 year and 10 months after fusion. However, the arteriovenous malformations still remained. Careful observation should be maintained continuously in the coming days. CONCLUSION: In arteriovenous malformations with destruction of the vertebral bodies, embolization and spinal fusion with segmental instrumentation may be necessary to relieve pain and prevent the progression of spinal deformity. Arteriovenous malformation should be considered in the diagnostic evaluation of a patient who has experienced vertebral collapse with no inflammatory signs.
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5/60. Spinal epidural hematoma caused by extradural arteriovenous malformation: a case report and review of the literature.

    About 330 cases of spinal epidural hematoma have been reported in the literature but few cases had pathologically proven extradural arteriovenous malformation. The authors report a case of spinal epidural hematoma caused by extradural arteriovenous malformation. The patient presented with a sudden onset of back pain followed by rapidly progressive neurological deficit. MRI was the procedure of choice for diagnosis of this lesion. Treatment was emergency surgical decompression. prognosis depends on the preoperative neurological deficit, operative interval and localization of the hematoma.
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6/60. Embolization of a spinal arteriovenous malformation: correlation between motor evoked potentials and angiographic findings: technical case report.

    OBJECTIVE AND IMPORTANCE: endovascular procedures for the treatment of spinal arteriovenous malformations place the spinal cord at risk of ischemia. This report illustrates the usefulness of motor evoked potentials (MEPs) in detecting functional changes within the spinal cord motor pathways during embolization of a spinal arteriovenous malformation under general anesthesia. CLINICAL PRESENTATION: A 28-year-old man presented with a history of progressive lower extremity numbness and weakness followed by bladder dysfunction. magnetic resonance imaging and angiography disclosed a T11-T12 spinal arteriovenous malformation. INTERVENTION: During the endovascular procedure, before injection of particles, the disappearance of MEPs from the tibialis anterior muscle led to prompt angiographic reevaluation, which disclosed the arrest of spinal blood flow secondary to radiculomedullary artery occlusion by the catheter. Embolization and catheter withdrawal were followed by temporary recovery of spinal blood flow and MEPs. A second arrest of spinal cord blood flow, caused by severe vasospasm of the feeding radiculomedullary artery, was documented by a control angiogram, and its functional relevance was revealed by a second disappearance of MEPs. The therapeutic effect of papaverine infusion and induced moderate hypertension was confirmed angiographically by complete reopacification of the anterior spinal artery and confirmed neurophysiologically by the complete recovery of MEPs. At the end of the procedure, no additional neurological deficits were noted. CONCLUSION: During spinal cord embolization, MEPs may play a critical role in early detection of spinal cord dysfunction by aiding in the prevention of damage to the spinal cord as well as by predicting the clinical outcome.
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keywords = muscle
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7/60. Congenital arteriovenous malformations of the face. Report of a case.

    arteriovenous malformations (AVM) are uncommon vascular abnormalities which can be either developmental defects present from birth, or acquired ones, secondary to trauma. They may cause local swelling and discomfort, and also may present a potential for severe bleeding. Traumatic AVM are usually single fistulous channels, whereas congenital lesions are most often multiple interconnections of vessels of varying caliber that result in a diffuseness which renders clini-al delineation and treatment difficult. The nature of the lesion is determined by the stage of development of the vascular system during which it develops. Congenital lesions range from finely channelled capillary hemangiomas to large-bore, high-flow A-V fistulas. Most lesions are not observed at birth, but become manifest later in life; minor trauma and endocrine change are implicated as the stimuli for the initiation of hemodynamic activity. The diagnosis is clinical, with confirmation by arteriography. The treatment has not been completely satisfactory in all cases. Surgical intervention has been used most often, but newer techniques include cryotherapy and muscle embolization.
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keywords = muscle
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8/60. A child with neurofibromatosis-1 and a lumbar epidural arteriovenous malformation.

    A 10-year-old child with neurofibromatosis-1 was evaluated for progressive lumbar scoliosis, back pain, and foot numbness. magnetic resonance imaging showed several lumbar intraspinal and extraspinal masses consistent with neurofibromas. The mass at L3-L5 compressed the thecal sac and was thought to be the source of the symptoms. On operative exploration, a lumbar epidural arteriovenous malformation was found, which was removed in its entirety. The child's back pain and foot numbness resolved. Epidural arteriovenous malformations in patients with neurofibromatosis-1 are rare and have been reported only in the cervical spine. Our finding of a lumbar epidural arteriovenous malformation in a child with neurofibromatosis-1 demonstrates that vascular anomalies can be present throughout the spine of patients with neurofibromatosis-1 and should be considered in the differential diagnosis of any neurofibromatosis-1-related epidural mass.
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9/60. Type IV spinal arteriovenous malformation in association with familial pulmonary vascular malformations: case report.

    OBJECTIVE AND IMPORTANCE: Type IVc arteriovenous malformations (AVMs) of the spinal cord consist of multiple high-flow feeding vessels, and they often present a challenging management situation. Their location is intradural and extramedullary, and they are rare malformations that are difficult to treat owing to the risk of thrombosis of the anterior spinal artery. The authors report a case of Type IVc spinal AVM in a patient with a family history of three siblings with pulmonary AVMs. Spinal AVMs have been reported to be associated with inherited syndromes such as familial cutaneous hemangiomas and Kartagener's syndrome, but an association with pulmonary AVMs has not previously been described. CLINICAL PRESENTATION: A 27-year-old man presented with sudden onset of occipital headache with cervical radiation while weightlifting. Results of computed tomography of the brain were normal, but lumbar puncture revealed a subarachnoid hemorrhage. The patient had a 1-year history of a neurogenic bladder and exhibited marked left calf muscle wasting. INTERVENTION: The patient underwent spinal magnetic resonance imaging, which revealed the AVM in the conus region. Selective spinal angiography was performed for diagnostic purposes. A laminectomy was performed, and the vessels feeding the AVM were clipped, as was the fistula. CONCLUSION: The patient remained neurologically stable, and angiography confirmed obliteration of the AVM. This is the first case report of a patient with a spinal AVM who had multiple siblings with pulmonary malformations or AVMs.
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keywords = muscle
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10/60. Congenital macroglossal angiodysplasia ("Lymphangioendotheliomatosis").

    A case of congenital lingual angiodysplasia with macroglossia in a 5-year-old girl is presented. A diffusely enlarged tongue was present at birth and continued to grow as the child aged. It was accompanied by impaired speech, difficulty in eating and breathing, and sleep apnea, necessitating surgical intervention. The fundamental lesion represents a complex vascular malformation of the lymphangioma-hemangioma type, involving extensively the deep musculature of the tongue. Multifocal and multicentric cavernous lymphangioma-like and hemangioma-like areas merge with benign angioendotheliomatous-like foci in a background of variable muscle degeneration and marked fibrosis. Neither a borderline nor an overtly malignant vasoformative neoplasm was present. Because of its distinctively widespread, multicentric intramuscular distribution, this lesion may be construed as a diffuse variant of lingual lymphangioma-hemangioma malformation, closely resembling a previously described case of macroglossal lymphangioendotheliomatosis.
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keywords = muscle, back
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