Abstract:ASCULAR lesions of the spinal cord are a well-recognized entity, having been comprehensively reviewed by Elsberg, 3 Wyburn-Mason, 6 and more recently by Odom. 4 Epidural vascular lesions are commonly hematomas or hemangiomas. Arteriovenous fistulas involving the vertebral vessels in the neck have occasionally been reported? ,2 .~ Nearly all of these fistulas have resulted from penetrating neck injuries, and the venous component has not primarily involved the epidural space. To our knowledge, only a single case… Show more
ObjectOur understanding of spinal extradural arteriovenous fistulas (eAVFs) is relatively limited. In this study the authors aimed to provide the demographics, natural history, and treatment results of these rare lesions.MethodsThe authors performed a pooled analysis of data in the PubMed database through December 2012. Individualized patient data were extracted to elucidate demographic, clinical, and angioarchitectural features of spinal eAVFs as well as outcomes following different treatment strategies.ResultsInformation on 101 patients was extracted from 63 eligible studies. The mean patient age was 45.9 years, and there was no significant overall sex predilection. Only 3% of the lesions were incidental, whereas 10% occurred in patients who had presented with hemorrhage. None of the 64 patients with at least 1 month of untreated follow-up sustained a hemorrhage over a total of 83.8 patient-years. Patients with lumbosacral eAVFs were significantly older (mean age 58.7 years, p < 0.0001), were significantly more often male (70% male, p = 0.02), had significantly worse presenting Aminoff-Logue motor and bladder scores (p = 0.0008 and < 0.0001, respectively), and had the greatest prevalence of lesions with intradural venous drainage (62% of cases, p < 0.0001). Neurofibromatosis Type 1 (30% of cases, p < 0.0001) and subarachnoid hemorrhage (9% of cases, p = 0.06) were associated with and exclusively found in patients with cervical eAVFs. The overall complete obliteration rate was 91%. After a mean follow-up of 1.7 years, the clinical condition was improved in 89% of patients, the same in 9%, and worse in 2%. Obliteration rates and outcome at follow-up did not significantly differ between surgical and endovascular treatment modalities.ConclusionsSpinal eAVFs are rare lesions with a low risk of hemorrhage; they cause neurological morbidity as a result of mass effect and/or venous hypertension. Their treatment is associated with a high rate of complete obliteration and improvement in preoperative symptoms.
ObjectOur understanding of spinal extradural arteriovenous fistulas (eAVFs) is relatively limited. In this study the authors aimed to provide the demographics, natural history, and treatment results of these rare lesions.MethodsThe authors performed a pooled analysis of data in the PubMed database through December 2012. Individualized patient data were extracted to elucidate demographic, clinical, and angioarchitectural features of spinal eAVFs as well as outcomes following different treatment strategies.ResultsInformation on 101 patients was extracted from 63 eligible studies. The mean patient age was 45.9 years, and there was no significant overall sex predilection. Only 3% of the lesions were incidental, whereas 10% occurred in patients who had presented with hemorrhage. None of the 64 patients with at least 1 month of untreated follow-up sustained a hemorrhage over a total of 83.8 patient-years. Patients with lumbosacral eAVFs were significantly older (mean age 58.7 years, p < 0.0001), were significantly more often male (70% male, p = 0.02), had significantly worse presenting Aminoff-Logue motor and bladder scores (p = 0.0008 and < 0.0001, respectively), and had the greatest prevalence of lesions with intradural venous drainage (62% of cases, p < 0.0001). Neurofibromatosis Type 1 (30% of cases, p < 0.0001) and subarachnoid hemorrhage (9% of cases, p = 0.06) were associated with and exclusively found in patients with cervical eAVFs. The overall complete obliteration rate was 91%. After a mean follow-up of 1.7 years, the clinical condition was improved in 89% of patients, the same in 9%, and worse in 2%. Obliteration rates and outcome at follow-up did not significantly differ between surgical and endovascular treatment modalities.ConclusionsSpinal eAVFs are rare lesions with a low risk of hemorrhage; they cause neurological morbidity as a result of mass effect and/or venous hypertension. Their treatment is associated with a high rate of complete obliteration and improvement in preoperative symptoms.
“…5,13 It is possible that these extradural AVM/ AVFs arise as congenital or acquired lesions; interestingly, the patient in Case 4 developed a fistula 30 years following laminectomy, supporting the possibility that these lesions are acquired. Purely extradural AVFs have been associated with penetrating and nonpenetrating trauma, neurofibromatosis, 7 and other more extensive vascular malformations.…”
ObjectAlthough nontraumatic spinal arteriovenous malformations and fistulas (AVMs and AVFs) restricted to the epidural space are rare, they can lead to significant neurological morbidity. Careful diagnostic imaging is essential to their detection and the delineation of the pathological anatomy. Aggressive endovascular and open operative treatment can provide arrest and reversal of neurological deficits.MethodsThe authors report on 6 cases of extradural AVMs/AVFs causing progressive myelopathy. Clinical findings, diagnostic evaluation, treatment, and outcome are discussed. Special consideration is given to the anatomy of the lesions and the operative techniques used to treat them. A review of the literature concerning extradural vascular malformations is also presented.ResultsAll 6 cases of extradural AVMs had an extradural fistulous location with intradural medullary venous drainage. These cases illustrate progressive myelopathy through cord venous congestion (hypertension) that can be caused by an extradural nidus or fistula. In 4 cases, a large epidural lake was identified on angiography. At surgery, the epidural lake was obliterated and medullary drainage interrupted. All patients had stabilization of their neurological deficits and successful obliteration of the AVM/AVF was obtained.ConclusionsExtradural AVMs and AVFs are a poorly described entity with published clinical experience limited to sporadic case reports and small series. Although these lesions have a purely extradural location of arteriovenous shunting and early venous drainage, they can be responsible for acute and progressive neurological symptoms similar to those caused by their dural-based intradural counterparts. With careful imaging recognition of the pathological anatomy, surgical and endovascular techniques can be used for the treatment of extradural AVMs affording effective and durable obliteration with stabilization or reversal of neurological symptoms. Venous drainage directly correlates the pathologic mechanisms of presentation. Specific attention must be paid intraoperatively to the epidural lake common to both variants so that recurrence is avoided.
“…In 1966, Hoffman was the first to report EESAVM confirmed by angiography [18]. The so called modern era in the treatment of spinal vascular malformation began in 1969 with Krayenbuhl and Yasargil and the publication of their microsurgical techniques, based heavily on the use of the operating microscope and bipolar cautery [19][20][21].…”
Section: Historical Perspectivementioning
confidence: 99%
“…Most reported cases (62.5%) treated by surgical operation or combined with embolization have no residual neurological symptoms (Table 1). Harry reported a seventeen years old male with EESAVM in 1967, he was died of introduction of anaesthesia rather than the EESAVM itself [18], except this case all cases postoperative neurological syndromes had improvement. After 1990, all the reported cases treated by surgical operation can walk post-operation with or without support; no patient neurological symptoms get worse than pre-operation.…”
Section: Treatment and Outcomesmentioning
confidence: 99%
“…Unruptured EESAVM may symptomless, or present as protracted, progressive neurological decline [18,23,33,34]. Ruptured EESAVM may present acute back or thoracic pain subsequently with paraplegia [17,[24][25][26][27][28][29]31,32,36].…”
Section: Clinical Presentation and Diagnosismentioning
The incidence of exclusively epidural spinal arteriovenous malformation (EESAVM) is extremely low and there are only a few case-reports in literature. Early, correct recognition of the pathology is mandatory to halt the progression of the disease and minimize permanent spinal cord injury. This review depicts EESAVM's characteristics, from the aspect of pathophysiology, clinical presentation, treatment strategies, and outcome. EESAVMs are located entirely in the epidural space and fed by radicular vessels or segmental arteries. The nidus of EESAVM purely locates in the spinal epidural space. The drainage of nidus flows into the epidural venous plexus or intradural vein. The retrograde blood flow from the AVM results in a diminished intramedullary blood flow and symptoms due to spinal cord ischemia and myelopathy. There is no gender predilection (male 52.9%), age distribution by the time of diagnosis shows most cases are younger than 20-year (64.7%), most cases present as spontaneous epidural hematoma (64.7%), and the majority of those lesions are located on cervicothoracic junction or upper thoracic segment (75%). Generally, the clinical presentation of EESAVM is slighter than intradural/intramedullary AVMs. Unruptured EESAVM may symptomless, or present as protracted, progressive neurological decline. Ruptured EESAVM may present acute back or thoracic pain subsequently with paraplegia. Epidural haemorrhage is common and urgent condition for EESAVM. Up to now, spinal angiography remains the gold standard and the first choice for diagnosis and characterization of spinal vascular lesion. The best management for EESAVM is still surgical operation. Prompt diagnosis and emergency surgical treatment are crucial. Long-term functional prognosis of EESAVM is good, but delayed surgical operation leave residual symptoms.
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