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1/1124. Retinocephalic vascular malformation: case report.

    A 12-year-old boy who presented with unilateral blindness and exophthalmos was found to have retinocephalic vascular malformations (Bonnet-Dechaumme-Blanc syndrome or Wyburn-Mason Syndrome). The ophthalmic, neurological and radiological findings of this rare syndrome are discussed.
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2/1124. Percutaneous and combined percutaneous and intralesional Nd:YAG-laser therapy for vascular malformations.

    The numerous types of vascular abnormality are classified in groups according to their pathological and anatomical features. We present case histories of 2 patients who had vascular malformations of the face since birth or early childhood. Application methods, side-effects and complications of percutaneous and intra-lesional Nd:YAG-laser therapy are reviewed for these patients. A 54-year-old woman was treated percutaneously with the Nd: YAG-laser at 1064 nm, with 20 30 W, cw 1-5 s pulses and 2 - 3 mm spot size. A 59-year-old woman was treated with the combined percutaneous and intralesional laser therapy with 30 W, cw 1-5 s pulses and 2-3 mm spot size. In both cases, percutaneous or combined percutaneous and intra-lesional Nd: YAG-laser application resulted in a significant shrinking of the lesion. The Nd:YAG-laser radiation at 1064 nm presents an effective treatment of vascular malformations due to its deep penetration into the tissue. No standardized guidelines for Nd: YAG-laser therapy exist and the treatment parameters should be chosen individually according to the type of vascular malformation.
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3/1124. Scrotal arteriovenous malformation and its preoperative embolization.

    arteriovenous malformations of the scrotum are extremely rare. A case of scrotal arteriovenous malformation and its preoperative embolization in a child is presented.
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4/1124. Inferior division third nerve paresis from an orbital dural arteriovenous malformation.

    Isolated inferior division third nerve palsies are rare. The authors report a patient with an isolated, pupil-involving inferior division third cranial nerve palsy from an intraorbital dural arteriovenous malformation. Despite spontaneous thrombosis of the malformation, the third nerve palsy persisted. To the authors' knowledge, this is the first case report of an inferior division third nerve palsy caused by an orbital dural arteriovenous malformation.
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5/1124. Venous malformation of the glans penis: efficacy of treatment with neodymium:yttruim-aluminum-garnet laser.

    OBJECTIVES: Vascular anomalies in the urogenital tract are very rare and those located in glans penis are the most uncommon. There is some controversy concerning their nomenclature, diagnosis and treatment. We present an analysis of our experience and a critical review of the literature. methods: Three cases of vascular lesions in the glans penis concordant with venous malformations were diagnosed at our center in 1996. The patients were 22, 20, and 12 years old, and attended our center for aesthetic reasons only. Even though two of the lesions were large, treatment with neodymium:yttrium-aluminium-garnet (Nd:YAG) laser irradiation was used. RESULTS: The outcome of treatment was satisfactory and no signs of recurrence were observed at follow-up examinations after a minimum of 18 months. CONCLUSIONS: Until recently, surgery has been the classical therapeutic approach, and only a few cases treated with the Nd:YAG laser have been reported. However, in our opinion, this should be the first choice treatment for this anomaly, given its efficacy, cosmetic results, and low morbidity.
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6/1124. 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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7/1124. 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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8/1124. Intraventricular hemorrhage as a false localizing sign of a thoracolumbar arteriovenous malformation: case report.

    BACKGROUND: Spinal arteriovenous malformation (SAVM) is a relatively rare disease characterized by a high incidence of intramedullary and subarachnoid haemorrhage. When the hemorrhage is profuse and the SAVM is in the cervical region the symptoms (disturbance of consciousness, papilledema, cranial nerve palsies, and convulsions) may be so severe and rapid in their onset that they may be mistaken for intracranial hemorrhage. We report here on a patient with a SAVM at T10-12, which bled intracranially, mainly intraventricularly, and resulted first in respiratory arrest and unconsciousness. CASE DESCRIPTION: The patient had been well until he was 28 years old when, during intercourse, he suffered a terrible headache and suddenly lost consciousness, with a transient respiratory arrest. He was also noted to have right hemiparesis. A computed tomography scan demonstrated intraventricular hemorrhage. After a 24-hour period of artificial ventilation the patient regained consciousness and the right arm paresis completely recovered, but a gradual worsening of the motor function of the left leg developed. Digital subtraction angiography did not demonstrate any intracranial source of bleeding, whereas spinal angiography revealed a SAVM located at the medullary cone, which was totally removed by surgery. CONCLUSION: The case reported here raises several important issues. First, the advisability of spinal magnetic resonance imaging in the investigation of intraventricular (and subarachnoid) hemorrhage in patients with no demonstrable intracranial source. Secondly, the benefits of early diagnosis and reestablishment of the spinal cord circulation before the onset of thrombosis and the progressive phase of myelopathy. Finally, the necessity of complete obliteration and treatment of SAVMs even in patients with fixed neurologic deficits, because rebleeding of lower thoracic or lumbar SAVMs can lead to impairment at a higher level with severe or lethal consequences.
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9/1124. Arteriovenous malformation of mesosalpinx associated with a 'vanishing' ectopic pregnancy: diagnosis with three-dimensional color power angiography.

    We describe two cases of pelvic arteriovenous malformation diagnosed with the aid of three-dimensional color power angiography. In both cases, beta-human chorionic gonadotropin (beta-hCG) increased to significant levels (8413 and 1560 mIU/ml, respectively); however, neither an intrauterine nor an adnexal gestational sac could be found. In each case, we observed an adnexal mass with several tortuous areas exhibiting abundant turbulent flow. The diagnosis of arteriovenous malformation was made and further assessment by three-dimensional color power angiography and magnetic resonance imaging (MRI) was carried out. The complex vascular anatomy of arteriovenous malformation, including its feeding vessels and drainage, was clearly depicted by three-dimensional color power angiography and correlated well with magnetic resonance angiography. Levels of beta-hCG decreased in subsequent tests, and eventually became negative 2-3 months later without and intervention. We believe that an involutional ectopic pregnancy induced the rapid growth of the arteriovenous malformations within the mesosalpinx. Three-dimensional color power angiography can be performed quickly and easily, using existing ultrasound equipment. It improves our understanding of complicated vasculature, and thus is a useful adjunct to two-dimensional and color Doppler ultrasound in the diagnosis of arteriovenous malformation.
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10/1124. Surgical management of angiographically occult spinal dural arteriovenous fistulae (type I spinal arteriovenous malformations): three technical case reports.

    OBJECTIVE AND IMPORTANCE: Spinal dural arteriovenous fistulae (Type I spinal arteriovenous malformations [AVMs]) have been recognized as a treatable cause of progressive myelopathy. The diagnosis and characterization of these lesions rest heavily on spinal angiography. CLINICAL PRESENTATION: We present three patients whose clinical presentations were consistent with a spinal dural AVM but whose spinal angiographic results were negative. INTERVENTION: The surgical treatment of these angiographically occult spinal AVMs is described. In retrospect, in each case, the feeding vessel to the AVM was injected but not seen. CONCLUSION: Some spinal dural arteriovenous fistulae will be angiographically occult. If the clinical and radiographic presentations strongly suggest the presence of an arteriovenous fistula, surgical exploration should be considered.
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