Clinical presentation and treatment outcomes of spinal epidural arteriovenous fistulas

OBJECTIVESpinal epidural arteriovenous fistulas (SEDAVFs) constitute a rare but treatable cause of vascular myelopathy and are a different subtype from the more common Type I spinal dural AVFs. The purpose of this study was to review a consecutive series of SEDAVFs from a single institution and report on the clinical presentations, functional status, and treatment outcomes.METHODSThe authors identified all SEDAVFs treated at their institution from 2005 to 2015. SEDAVFs were defined as spinal AVFs in which the fistulous connection occurred in the epidural venous plexus. The clinical presentation, functional status, immediate treatment outcomes, and long-term neurological outcomes were analyzed.RESULTSTwenty-four patients with SEDAVFs were included in this study. The patients' mean age at presentation was 66.9 years. The most common presenting symptoms were pain and numbness (22 patients, 91.7%), followed by lower-extremity weakness (21 patients, 87.5%). The mean duration of symptoms prior to diagnosis was 11.8 months. Eighteen patients (75.0%) were treated with endovascular therapy alone, 4 (16.7) with surgery, and 2 (8.3%) with a combination of techniques. There was 1 major treatment-related complication (4.2%). Fifteen patients (62.5%) had improvement in disability, and 12 patients (54.5%) had improvement in sensory symptoms.CONCLUSIONSSEDAVFs often present with lower-extremity motor dysfunction and sensory symptoms. With the availability of newer liquid embolic agents, these lesions can be effectively treated with endovascular techniques. Surgery is also effective at treating these lesions, especially in situations where endovascular embolization fails or is not safe and in patients presenting with mass effect from compressive varices.

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Clinical and angioarchitectural characteristics of spinal vascular malformations of the cervical spine

Journal of Neurosurgery Spine ◽

10.3171/2019.11.spine19798 ◽

2020 ◽

Vol 32 (5) ◽

pp. 755-762 ◽

Cited By ~ 1

Author(s):

Waleed Brinjikji ◽

Elisa Colombo ◽

Giuseppe Lanzino

Keyword(s):

Cervical Spine ◽

Lower Extremity ◽

Clinical Presentation ◽

Vascular Malformations ◽

Case Series ◽

Acute Onset ◽

Lower Extremity Weakness ◽

Arteriovenous Fistulas ◽

Spinal Vascular Malformations ◽

Interventional Neuroradiologist

OBJECTIVEVascular malformations of the cervical spine are exceedingly rare. To date there have been no large case series describing the clinical presentation and angioarchitectural characteristics of cervical spine vascular malformations. The authors report their institutional case series on cervical spine vascular malformations diagnosed and treated at their institution.METHODSThe authors retrospectively reviewed all patients with spinal vascular malformations from their institution from January 2001 to December 2018. Patients with vascular malformations of the cervical spine were included. Lesions were characterized by their angioarchitectural characteristics by an interventional neuroradiologist and endovascular neurosurgeon. Data were collected on clinical presentation, imaging findings, treatment outcomes, and long-term follow-up. Descriptive statistics are reported.RESULTSOf a total of 213 patients with spinal vascular malformations, 27 (12.7%) had vascular malformations in the cervical spine. The mean patient age was 46.1 ± 21.9 years and 16 (59.3%) were male. The most common presentations were lower-extremity weakness (13 patients, 48.1%), tetraparesis (8 patients, 29.6%), and lower-extremity sensory dysfunction (7 patients, 25.9%). Nine patients (33.3%) presented with hemorrhage. Fifteen patients (55.6%) had modified Rankin Scale scores of 0–2 at the time of diagnosis. Regarding angioarchitectural characteristics, 8 patients (29.6%) had intramedullary arteriovenous malformations (AVMs), 5 (18.5%) had epidural arteriovenous fistulas (AVFs), 4 (14.8%) had paraspinal fistulas, 4 (14.8%) had mixed epidural/intradural fistulas, 3 (11.1%) had perimedullary AVMs, 2 (7.4%) had dural fistulas, and 1 patient (3.7%) had a perimedullary AVF.CONCLUSIONSThis retrospective study of 27 patients with cervical spine vascular malformations is the largest series to date on these lesions. The authors found substantial angioarchitectural heterogeneity with the most common types being intramedullary AVMs followed by epidural AVFs, paraspinal fistulas, and mixed intradural/extradural fistulas. Angioarchitecture dictated the clinical presentation as intradural shunts were more likely to present with hemorrhage and acute onset myelopathy, while dural and extradural shunts presented as either incidental lesions or gradually progressive congestive myelopathy.

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Open and endovascular treatment of spinal dural arteriovenous fistulas: a 10-year experience

Journal of Neurosurgery Spine ◽

10.3171/2016.9.spine16394 ◽

2017 ◽

Vol 26 (4) ◽

pp. 519-523 ◽

Cited By ~ 20

Author(s):

Matthew J. Koch ◽

Christopher J. Stapleton ◽

Pankaj K. Agarwalla ◽

Collin Torok ◽

John H. Shin ◽

...

Keyword(s):

Vascular Malformations ◽

Management Strategies ◽

Case Series ◽

Endovascular Embolization ◽

Definitive Treatment ◽

Type I ◽

Arteriovenous Fistulas ◽

Presenting Symptoms ◽

Dural Arteriovenous Fistulas ◽

Spinal Dural Arteriovenous Fistulas

OBJECTIVE Vascular malformations of the spine represent rare clinical entities with profound neurological implications. Previously reported studies on management strategies for spinal dural arteriovenous fistulas (sDAVFs) appeared before the advent of modern liquid embolic agents. Authors of the present study review their institutional experience with endovascularly and surgically treated sDAVFs. METHODS The authors performed a retrospective, observational, single-center case series on sDAVFs treated with endovascular embolization, microsurgical occlusion, or both between 2004 and 2013. The mode, efficacy, and clinical effect of treatment were evaluated. RESULTS Forty-seven patients with spinal arteriovenous malformations were evaluated using spinal angiography, which demonstrated 34 Type I sDAVFs (thoracic 20, lumbar 12, and cervical 2). Twenty-nine of the patients (85%) were male, and the median patient age was 63.3 years. Twenty patients underwent primary endovascular embolization (16 Onyx, 4 N-butyl cyanoacrylate [NBCA]), and 14 underwent primary surgical clipping. At a mean follow-up of 36 weeks, according to angiography or MR angiography, 5 patients treated with endovascular embolization demonstrated persistent arteriovenous shunting, whereas none of the surgically treated patients showed lesion persistence (p = 0.0237). Thirty patients (88%) experienced some resolution of their presenting symptoms (embolization 17 [85%], surgery 13 [93%], p = 1.00). CONCLUSIONS Microsurgical occlusion remains the most definitive treatment modality for sDAVFs, though modern endovascular techniques remain a viable option for the initial treatment of anatomically amenable lesions. Treatment of these lesions usually results in some clinical improvement.

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Late diagnosis of spinal dural arteriovenous fistulas resulting in severe lower-extremity weakness: a case series

The Spine Journal ◽

10.1016/j.spinee.2013.08.029 ◽

2015 ◽

Vol 15 (6) ◽

pp. e39-e44 ◽

Cited By ~ 21

Author(s):

Ivelin Iovtchev ◽

Nurith Hiller ◽

Yona Ofran ◽

Isabella Schwartz ◽

Jose Cohen ◽

...

Keyword(s):

Lower Extremity ◽

Case Series ◽

Late Diagnosis ◽

Lower Extremity Weakness ◽

Arteriovenous Fistulas ◽

Dural Arteriovenous Fistulas ◽

Spinal Dural Arteriovenous Fistulas

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Lower Extremity Fibromuscular Dysplasia: Clinical Manifestations, Diagnostic Testing, and Approach to Management

Angiology ◽

10.1177/0003319716682121 ◽

2016 ◽

Vol 68 (8) ◽

pp. 722-727 ◽

Cited By ~ 5

Author(s):

Ellen Brinza ◽

Victoria Grabinski ◽

Sridevi Durga ◽

Sarah O’Connor ◽

Sandra L. Yesenko ◽

...

Keyword(s):

Lower Extremity ◽

Carotid Arteries ◽

Fibromuscular Dysplasia ◽

Clinical Presentation ◽

Diagnostic Testing ◽

Clinical Manifestations ◽

Single Center ◽

Iliac Arteries ◽

Presenting Symptoms ◽

Vascular Bed

Fibromuscular dysplasia (FMD), a disease well described in the renal and cerebrovascular circulations, also manifests in the lower extremity (LE) arteries. This study reports on the clinical presentation, imaging findings, and treatment of patients with LE FMD seen at a single center. Over a 7-year span, 100 of 449 patients with FMD had imaging of the LE arteries, of which 62 were found to have LE FMD (13.8% of the entire FMD cohort including patients with and without LE imaging). The majority of patients were women (96.8%), with an average age of 52 ± 11.3 years at the time of diagnosis. All patients had FMD present in another vascular bed, most commonly in the renal (80.6%) and extracranial carotid arteries (79.0%). Most patients had multifocal FMD (95.2%) and bilateral LE disease (69.4%), with the external (87.1%), common (19.4%), and internal (11.3%) iliac arteries most commonly affected. Presenting symptoms of LE involvement included claudication (22.6%), atypical leg symptoms (14.5%), and dissection (6.5%), but most patients were asymptomatic (71.0%). Nearly all patients were managed conservatively (98.4%) and only 1 patient required intervention.

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Pediatric intracranial dural arteriovenous fistulas: age-related differences in clinical features, angioarchitecture, and treatment outcomes

Journal of Neurosurgery Pediatrics ◽

10.3171/2016.5.peds15740 ◽

2016 ◽

Vol 18 (5) ◽

pp. 602-610 ◽

Cited By ~ 9

Author(s):

Steven W. Hetts ◽

Parham Moftakhar ◽

Neil Maluste ◽

Heather J. Fullerton ◽

Daniel L. Cooke ◽

...

Keyword(s):

Treatment Outcomes ◽

Clinical Outcomes ◽

Clinical Presentation ◽

Neurological Deficits ◽

Older Children ◽

Arteriovenous Shunting ◽

Arteriovenous Fistulas ◽

Age Related ◽

End Of Treatment ◽

Dural Arteriovenous Fistulas

OBJECTIVE Intracranial dural arteriovenous fistulas (DAVFs) are rare in children. This study sought to better characterize DAVF presentation, angioarchitecture, and treatment outcomes. METHODS Children with intracranial DAVFs between 1986 and 2013 were retrospectively identified from the neurointerventional database at the authors' institution. Demographics, clinical presentation, lesion angioarchitecture, treatment approaches, angiographic outcomes, and clinical outcomes were assessed. RESULTS DAVFs constituted 5.7% (22/423) of pediatric intracranial arteriovenous shunting lesions. Twelve boys and 10 girls presented between 1 day and 18 years of age; boys presented at a median of 1.3 years and girls presented at a median of 4.9 years. Four of 8 patients ≤ 1 year of age presented with congestive heart failure compared with 0/14 patients > 1 year of age (p = 0.01). Five of 8 patients ≤ 1 year old presented with respiratory distress compared with 0/14 patients > 1 year old (p = 0.0021). Ten of 14 patients > 1 year old presented with focal neurological deficits compared with 0/8 patients ≤ 1 year old (p = 0.0017). At initial angiography, 16 patients harbored a single intracranial DAVF and 6 patients had 2–6 DAVFs. Eight patients (38%) experienced DAVF obliteration by the end of treatment. Good clinical outcome (modified Rankin Scale score 0–2) was documented in 77% of patients > 1 year old at presentation compared with 57% of patients ≤ 1 year old at presentation. Six patients (27%) died. CONCLUSIONS Young children with DAVFs presented predominantly with cardiopulmonary symptoms, while older children presented with focal neurological deficits. Compared with other pediatric vascular shunts, DAVFs had lower rates of angiographic obliteration and poorer clinical outcomes.

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Filum terminale arteriovenous fistulas: the role of endovascular treatment

Journal of Neurosurgery Spine ◽

10.3171/2013.4.spine12685 ◽

2013 ◽

Vol 19 (1) ◽

pp. 49-56 ◽

Cited By ~ 18

Author(s):

Ekachat Chanthanaphak ◽

Sirintara Pongpech ◽

Pakorn Jiarakongmun ◽

Chai Kobkitsuksakul ◽

Cuong Tran Chi ◽

...

Keyword(s):

Clinical Presentation ◽

Radicular Pain ◽

Venous Hypertension ◽

Sensory Loss ◽

Imaging Features ◽

Filum Terminale ◽

Related Complication ◽

Duration Of Symptoms ◽

Arteriovenous Fistulas

Object The authors describe the clinical presentation, imaging features, and management of patients presenting with filum terminale arteriovenous fistulas (FTAVFs) and the role of transarterial treatment in their management. Methods The authors retrospectively reviewed data obtained in 10 patients with FTAVFs diagnosed between January 1990 and December 2011. Results Most patients (70%) were male, and the age of the population ranged from 31 to 72 years (mean 58.2 years). Clinical presentation was progressive paraparesis and sensory loss in the lower extremities in 9 cases, back pain in 7, radicular pain in 3, bowel/bladder disturbance in 5, and impotence in 1. The duration of symptoms varied between 2 and 24 months. Initial MRI studies showed intramedullary increased T2 signal, swollen cord, and dilated perimedullary veins in all patients. One patient had syringomyelia, presumably caused by venous hypertension transmitted by the perimedullary venous system. Embolization was attempted in 7 patients and was curative in 6 patients. Surgery was performed in the other 4 patients in whom embolization was unsuccessful or deemed not feasible. There was no treatment-related complication in either group. Symptoms, venous congestion in the cord, and syringomyelia improved on follow-up in all patients. Conclusions Embolization should be considered the treatment of choice for FTAVFs and can effectively treat the majority of patients presenting with an FTAVF. In a smaller group of patients in whom the angioarchitecture is unfavorable, open surgery is recommended.

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Borden-Shucart Type I dural arteriovenous fistulas: clinical course including risk of conversion to higher-grade fistulas

Journal of Neurosurgery ◽

10.3171/2012.5.jns111257 ◽

2012 ◽

Vol 117 (3) ◽

pp. 539-545 ◽

Cited By ~ 23

Author(s):

Manish N. Shah ◽

James A. Botros ◽

Thomas K. Pilgram ◽

Christopher J. Moran ◽

DeWitte T. Cross ◽

...

Keyword(s):

Intracranial Hemorrhage ◽

Clinical Course ◽

Retrospective Chart Review ◽

Annual Rate ◽

Neurological Deficits ◽

Type I ◽

Arteriovenous Fistulas ◽

Presenting Symptoms ◽

Dural Arteriovenous Fistulas

Object The goal of this study was to determine the clinical course of Borden-Shucart Type I cranial dural arteriovenous fistulas (DAVFs) and to calculate the annual rate of conversion of these lesions to more aggressive fistulas that have cortical venous drainage (CVD). Methods A retrospective chart review was conducted of all patients harboring DAVFs who were seen at the authors' institution between 1997 and 2009. Twenty-three patients with Type I DAVFs who had available clinical follow-up were identified. Angiographic and clinical data from these patients were reviewed. Neurological outcome and status of presenting symptoms were assessed during long-term follow-up. Results Of the 23 patients, 13 underwent endovascular treatment for intolerable tinnitus or ophthalmological symptoms, and 10 did not undergo treatment. Three untreated patients died of unrelated causes. In those who were treated, complete DAVF obliteration was achieved in 4 patients, and palliative reduction in DAVF flow was achieved in 9 patients. Of the 19 patients without radiographic cure, no patient developed intracranial hemorrhage or nonhemorrhagic neurological deficits (NHNDs), and no patient died of DAVF-related causes over a mean follow-up of 5.6 years. One patient experienced a spontaneous, asymptomatic obliteration of a partially treated DAVF in late follow-up, and 2 patients experienced a symptomatic conversion of their DAVF to a higher-grade fistula with CVD in late follow-up. The annual rate of conversion to a higher-grade DAVF based on Kaplan-Meier cumulative event-free survival analysis was 1.0%. The annual rate of intracranial hemorrhage, NHND, and DAVF-related death was 0.0%. Conclusions A small number of Type I DAVFs will convert to more aggressive DAVFs with CVD over time. This conversion to a higher-grade DAVF is typically heralded by a change in patient symptoms. Follow-up vascular imaging is important, particularly in the setting of recurrent or new symptoms.

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Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea

Frontiers in Endocrinology ◽

10.3389/fendo.2020.610746 ◽

2021 ◽

Vol 11 ◽

Author(s):

Hyojung Park ◽

Min-Sun Kim ◽

Jiwon Lee ◽

Jung-Han Kim ◽

Byong Chang Jeong ◽

...

Keyword(s):

Children And Adolescents ◽

Treatment Outcomes ◽

Genetic Screening ◽

Clinical Presentation ◽

Medical Center ◽

Care Center ◽

Tertiary Care ◽

Screening Tests ◽

Laboratory Findings ◽

Presenting Symptoms

PurposePheochromocytoma (PCC) and paraganglioma (PGL) (PPGL) are rare neuroendocrine tumors, and data on managing these conditions in children and adolescents are lacking. The objective of this study was to demonstrate the clinical presentation and treatment outcomes in children and adolescents with PPGL in a single tertiary care center in Korea.MethodsThis retrospective study included 23 patients diagnosed with PCC (n = 14) and PGL (n = 9) before the age of 21 at Samsung Medical Center (from June 1994 to June 2019). We describe age, gender, family history, clinical characteristics, laboratory findings, pathologic findings, therapeutic approaches, and treatment outcomes.ResultsOf the 23 patients, 14 had PCC and nine had PGL. The median age at diagnosis was 16.8 years (range, 6.8–20.8 years). The common presenting symptoms were hypertension (n = 10), headache (n = 9), palpitation (n = 4), and sweating (n = 4). The plasma or 24-hour urine catecholamine and/or metabolite concentrations were markedly elevated in 22 patients with PPGL, but were normal in one patient with carotid body PGL. All tumors were visualized on computed tomography. Genetic tests were performed in 15 patients, and seven patients showed mutations in RET (n = 3), SDHB (n = 3), and VHL (n = 1). All patients underwent surgery, and complete excision was performed successfully. Three patients with metastasis underwent postoperative adjuvant therapy.ConclusionThis study suggests that pediatric PPGL tends to be extra-adrenal and bilateral and shows a higher potential for genetic mutations. Considering the hereditary predisposition of pediatric PPGL, genetic screening tests are strongly recommended, and lifelong follow-up is needed to detect recurrence and metastasis. Further research with a larger sample size and routine genetic screening is needed to better understand the genetic conditions and long-term prognosis of PPGL.

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Microsurgical versus endovascular treatment of spinal epidural arteriovenous fistulas with intradural venous drainage: a multicenter study of 81 patients

Journal of Neurosurgery Spine ◽

10.3171/2020.2.spine191432 ◽

2020 ◽

Vol 33 (3) ◽

pp. 381-391 ◽

Cited By ~ 2

Author(s):

Keisuke Takai ◽

Toshiki Endo ◽

Takao Yasuhara ◽

Toshitaka Seki ◽

Kei Watanabe ◽

...

Keyword(s):

Treatment Failure ◽

Endovascular Treatment ◽

Failure Rate ◽

Initial Treatment ◽

Venous Drainage ◽

Type I ◽

Arteriovenous Fistulas ◽

Multicenter Cohort ◽

Treatment Failure Rate ◽

Spinal Epidural

OBJECTIVESpinal arteriovenous shunts are rare vascular lesions and are classified into 4 types (types I–IV). Due to rapid advances in neuroimaging, spinal epidural AVFs (edAVFs), which are similar to type I spinal dural AVFs (dAVFs), have recently been increasingly reported. These 2 entities have several important differences that influence the treatment strategy selected. The purposes of the present study were to compare angiographic and clinical differences between edAVFs and dAVFs and to provide treatment strategies for edAVFs based on a multicenter cohort.METHODSA total of 280 consecutive patients with thoracic and lumbosacral spinal dural arteriovenous fistulas (dAVFs) and edAVFs with intradural venous drainage were collected from 19 centers. After angiographic and clinical comparisons, the treatment failure rate by procedure, risk factors for treatment failure, and neurological outcomes were statistically analyzed in edAVF cases.RESULTSFinal diagnoses after an angiographic review included 199 dAVFs and 81 edAVFs. At individual centers, 29 patients (36%) with edAVFs were misdiagnosed with dAVFs. Spinal edAVFs were commonly fed by multiple feeding arteries (54%) shunted into a single or multiple intradural vein(s) (91% and 9%) through a dilated epidural venous plexus. Preoperative modified Rankin Scale (mRS) and Aminoff-Logue gait and micturition grades were worse in patients with edAVFs than in those with dAVFs. Among the microsurgical (n = 42), endovascular (n = 36), and combined (n = 3) treatment groups of edAVFs, the treatment failure rate was significantly higher in the index endovascular treatment group (7.5%, 31%, and 0%, respectively). Endovascular treatment was found to be associated with significantly higher odds of initial treatment failure (OR 5.72, 95% CI 1.45–22.6). In edAVFs, the independent risk factor for treatment failure after microsurgery was the number of intradural draining veins (OR 17.9, 95% CI 1.56–207), while that for treatment failure after the endovascular treatment was the number of feeders (OR 4.11, 95% CI 1.23–13.8). Postoperatively, mRS score and Aminoff-Logue gait and micturition grades significantly improved in edAVFs with a median follow-up of 31 months.CONCLUSIONSSpinal epidural AVFs with intradural venous drainage are a distinct entity and may be classified as type V spinal vascular malformations. Based on the largest multicenter cohort, this study showed that primary microsurgery was superior to endovascular treatment for initial treatment success in patients with spinal edAVFs.

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Presentation and outcomes of patients with thoracic and lumbosacral spinal epidural arteriovenous fistulas: a systematic review and meta-analysis

Journal of NeuroInterventional Surgery ◽

10.1136/neurintsurg-2018-014203 ◽

2018 ◽

Vol 11 (1) ◽

pp. 95-98 ◽

Cited By ~ 3

Author(s):

Jun-Soo Byun ◽

Anderson Chun On Tsang ◽

Christopher Alan Hilditch ◽

Patrick Nicholson ◽

Yi-Bin Fang ◽

...

Keyword(s):

Systematic Review ◽

Clinical Symptoms ◽

Combined Treatment ◽

Venous Drainage ◽

Treatment Modalities ◽

Lumbosacral Spine ◽

Spinal Vascular Malformation ◽

Arteriovenous Fistulas ◽

Presenting Symptoms ◽

Spinal Epidural

Background and purposeThoracolumbar and sacral spinal epidural arteriovenous fistulas (SEDAVFs) are an increasingly recognized form of spinal vascular malformation. The purpose of this study was to perform a systematic review of the demographics, clinical presentation and treatment results of thoracolumbar SEDAVFs.Materials and methodsPubmed, Scopus and Web of Science databases were searched from January 2000 to January 2018 for articles on treatment of SEDAVFs. Pooled data of individual patients were analyzed for demographic and clinical features of SEDAVFs as well as treatment outcomes.ResultsThere were 125 patients from 11 studies included. Mean age was 63.5 years. There was a male sex predilection (69.6%). Sensory symptoms including pain or numbness were the most frequently presenting symptoms. Fistula location was the lumbosacral spine in 79.2% and the thoracic spine in 20.8%. Involvement of intradural venous drainage was more common than extradural venous drainage only (89.6% vs 10.4%). Of the 123 treated patients, endovascular therapy was performed in 67.5% of patients, microsurgery in 23.6%, and combined treatment in 8.9%. The overall complete obliteration rate was 83.5% and did not differ between groups. Clinical symptoms improved in 70.7% of patients, were stable in 25%, and worsened in 1.7% with no difference between treatment modalities.ConclusionsThoracic and lumbosacral SEDAVFs often present with symptoms secondary to congestive myelopathy or compressive symptoms. Both endovascular and microsurgical treatments were associated with high obliteration rates and good clinical outcomes.

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