We present a rare case of multiple spinal perimedullary arteriovenous fistulae associated with the Parkes-Weber (PW) syndrome. A 31-year-old male known to have the PW syndrome involving the left leg since birth, presented with a 7-month-history of progressive myelopathy of the lower extremities and dysfunction of the bladder and bowel. Myelography demonstrated dilated intradural vessels. Angiography demonstrated two distinct single hole perimedullary arteriovenous fistulae near the conus at two different metameres. They were supplied by the left posterior spinal artery. The patient was treated by transarterial embolisation using polyvinyl alcohol particles, which resulted in venous side occlusion of the fistulae. After the treatment, the patient developed transient worsening of the spasticity of the lower extremities, and was treated by heparinization. After heparinization, the patient partially recovered from the pre-embolisation status of his myelopathy. The follow-up angiogram one year after the embolisation demonstrated persistent obliteration of both fistulae. At long-term follow-up, the patient can ambulate without assistance and work as a farmer.
[Show abstract][Hide abstract] ABSTRACT: We describe a rare case of multiple arteriovenous fistulae of the spinal cord (SCAVF) in the same myelomer in a five-year-old boy. This case report consists of a trifocal SCAVF at the Th12 myelomeric level without communication between the three different fistulae. Two AVF were located posteriorly, bilateraly, in the spinal cord, fed by left and right posterior radiculopial arteries and one anteriorly in the anterior spinal axis. The venous drainage was independent for each lesion. The patient presents associated lesions characterized by cutaneous stain and inferior limb asymmetry. A metameric distribution is the explanation for the multiplicity of these lesions in a syndromic association related to Cobb syndrome. The patient was treated by transarterial embolization using glue with occlusion of the three different fistulae. The patient achieved a good improvement in neurological status.
[Show abstract][Hide abstract] ABSTRACT: Spinal cord arteriovenous shunts (SCAVSs) are either fistulas or niduses that can be separated in four different groups according to their localization and relationship to the dura. Paraspinal AVSs are located outside the spine and are responsible for neurological symptoms because of cord compression by ertatic veins, venous congestion or arterial steal. Epidural shunts are located in the epidural space and drain in epidural veins with secondary intradural congestion. Dural shunts are embedded in the dura, produce a cord venous myelopathy after draining through veins that either pierce the dura far from a nerve root or accompany a nerve root. Intradural shunts affect the cord, the roots or the filum. Additionally, they can be classified according to their potential relationships with genetics, vascular biological features and angiogenesis into genetic hereditary lesions (hereditary hemorrhagic telangiectasia), genetic non-hereditary lesions (multiple lesions with metameric links) and single lesions (AVMs or micro AVFs). MRI and MRA are able to visualise SCAVS early after the onset of clinical symptoms. The type of shunt and its localization may remain difficult to be precise. Angiography remains the gold standard for analysis of the anatomical, morphological and architectural features necessary for therapeutic decisions in both paediatric and adult populations. In our series, embolisation is chosen in first intention whatever the type of shunt responsible for the clinical symptoms and glue is preferably used. In paraspinal, dural or epidural arteriovenous shunts, the goal of treatment should be complete closure of the shunt. A complete cure by embolization is rather easily achieved in paraspinal lesions. Failure of endovascular therapy in dural or epidural shunts must bring the patient to surgery. The prognosis of most intradural shunts seems better than previously thought, even after haemorrhage. In intradural spinal cord arteriovenous shunts, embolisation targeted towards the portions of the malformation felt to be responsible for the symptoms (venous congestion) or pointing to the point of rupture (false aneurysms) of the malformation, allows restoration of a new hemodynamic equilibrium between the malformation and the cord itself. Such targeted treatment offers long-term stabilisation or improvement to patients suffering from SCAVSs and good protection against (re) haemorrhages, with an acceptable morbidity. Cure of the shunt is not imperative to obtain these satisfactory outcomes. The clinical results obtained by such management compare favourably with those obtained by neurosurgery.
European Journal of Radiology 07/2003; 46(3):221-32. DOI:10.1016/S0720-048X(03)00093-7 · 2.37 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We describe a 24-year-old woman who presented with twice previously unverified subarachnoid hemorrhages from the conus medullaris spinal arteriovenous malformation with Parkes-Weber-syndrome. Spinal MRI examination is considered to be necessary for the diagnosis of Klippel-Trenaunay-Weber syndrome. For diagnosis of the spinal cord arteriovenous malformation, it is indispensable to search carefully for the presence of accompanying lesions. Transarterial glue embolizationis effective for the management of the spinal vascular lesion.
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