Spinal Vascular Malformations Research Articles

Transarterial Embolization of Intracranial Dural Arteriovenous Fistulas Cognard Type V With Direct Drainage Into the Perimedullary Veins: Case Report and Literature Review.

Cognard type V fistula (CVF) is a rare type of dural arteriovenous fistula characterized by spinal perimedullary venous drainage. Owing to the lack of pathognomonic findings, misdiagnosis is common. Patients often undergo multiple spinal angiograms negative for spinal vascular malformations. Digital subtraction angiography is the gold standard diagnostic tool. The preferred treatment option is endovascular management with embolization through a transarterial, transvenous, or combined approach. Other options include open surgery, stereotactic radiosurgery, or a combination of both. The patient from case # 1 presented with progressive weakness and hypoesthesia in the bilateral lower extremities, with urinary and bowel incontinence. The DSA identified a CVF fed by the meningohypophyseal trunk and a draining perimedullary vein. Embolization with 0.1 ccs of Onyx-18 was performed with complete fistula occlusion. The patient from case # 2 developed bilateral lower extremity weakness, diffuse numbness, and urinary incontinence. The DSA showed a CVF fed by tributaries from the ascending pharyngeal artery and posterior meningeal artery branches of the V3 segment, draining into a perimedullary vein. Embolization with 0.3 cc of Onyx-18 was performed with 100% occlusion of the fistula. A 1-year follow-up angiogram confirmed complete fistula occlusion. Both patients consented to the procedure. Even if a patient only presents symptoms of myelopathy, CVF should be considered. Herein, we present 2 cases of CVF with direct drainage into the perimedullary veins which presented exclusively with myelopathy syndrome and describe treatment with trasarterial embolization with Onyx.

Case Report: Percutaneous intercostal artery catheterization to treat a difficult-to-access spinal dural arteriovenous fistula

Spinal dural arteriovenous fistulas (SDAVF) are a subtype of spinal arteriovenous malformation (AVM). Although rare, SDAVF are the most common spinal vascular malformations and have potential devastating neurologic consequences, sometimes irreversible. Originally, they were treated with surgery alone, however endovascular treatment of SDAVF has evolved recently with good results and the understanding of the anatomy plays a key role. The feeder vessel of the AV shunt is supplied by the radiculomedullary or radiculopial artery, the shunt is usually located within the root sleeve of the dura and the SDAVF is drained by the medullary vein via retrograde flow. Generally the endovascular approach uses the catheterization of the segmental arteries; however, aortic thoracolumbar aneurysms can render endovascular access difficult and risky. We present a case where an SDAVF in woman with aneurysmal dilation of the descending aorta and a mural thrombus was successfully treated through percutaneous catheterization of intercostal artery.

Diagnostic, clinical management, and outcomes in patients with spinal dural arteriovenous fistula.

Spinal dural arteriovenous fistulas (SDAVFs) are rare spinal vascular malformations, but account for 70 to 80% of all spinal arteriovenous malformations. SDAVFs can be treated either surgically or endovascularly, with surgical treatment appearing to lead to higher closure rates. Our aim was to analyze the demographic data, diagnostic history, treatment characteristics and clinical short- and long-term outcomes. The medical records of 81 patients who underwent surgical (n = 70, 86.4%) and endovascular (n = 11, 13.6%) treatment for SDAVF at a university hospital between 2002 and 2023 were retrospectively analyzed. SDAVF was observed more frequently in men than women (61, 75.3% vs. 20, 24.7%) with a mean age of 63.5 ± 12.7 years and a mean duration of symptoms to diagnosis of 12.0 ± 12.8 months. The most common first symptom was gait disturbance (36, 44.4%), followed by sensory disturbance (24, 29.6%). The location of the fistula point was most common in the lower thoracic region (36, 44.5%), followed by the lumbar region (23, 28.4%). Incomplete or failed occlusion of the fistula occurred in 8 patients (9.9%), with 6 patients (7.4%) undergoing further treatment either surgically or endovascularly. Treatment- or hospital-related complications were observed in 16 patients (19.8%). A single-level laminectomy was the most common approach (31, 44.3%), followed by single-level hemilaminectomy (28, 40.0%), and unilateral interlaminar fenestration (11, 15.7%). Back pain or radiculopathy was observed in 58% of patients (47/81) pre-treatment and had already decreased to 24.7% at hospital discharge (p < 0.001). No significant differences were observed in sensory disturbances (p = 0.681). The median of American Spinal Injury Association motor score (ASIA-MS) was 94 [82.5-100] at admission, 98 [86.5-100] at hospital discharge, 100 [90-100] at the first, second, and third follow-up (p = 0.019). The median modified Aminoff-Logue scale (mALS) was 5 [2-7] at admission, 3 [1-6] at hospital discharge, 2 [1-5] at the first follow-up, 2 [0.5-5] at the second follow-up and 2 [1-7] at the third follow-up (p = 0.006). SDAVF occurs predominantly in men in the 6th decade of life and can be safely and effectively treated surgically and endovascularly, improving symptoms such as pain and motor deficits, gait disturbances as well as bowel and bladder dysfunction, but not sensory disturbances.

Application of Spinal Subtraction and Bone Background Fusion CTA in the Accurate Diagnosis and Evaluation of Spinal Vascular Malformations.

Accurate pretreatment diagnosis and assessment of spinal vascular malformations using spinal CTA are crucial for patient prognosis, but the postprocessing reconstruction may not be able to fully depict the lesions due to the complexity inherent in spinal anatomy. Our purpose was to explore the application value of the spinal subtraction and bone background fusion CTA (SSBBF-CTA) technique in precisely depicting and localizing spinal vascular malformation lesions. In this retrospective study, patients (between November 2017 and November 2022) with symptoms similar to those of spinal vascular malformations were divided into diseased (group A) and nondiseased (group B) groups. All patients underwent spinal CTA using Siemens dual-source CT. Multiplanar reconstruction; routine bone subtraction, and SSBBF-CTA images were obtained using the snygo.via and ADW4.6 postprocessing reconstruction workstations. Multiple observers researched the following 3 aspects: 1) preliminary screening capability using original images with multiplanar reconstruction CTA, 2) the accuracy and stability of the SSBBF-CTA postprocessing technique, and 3) diagnostic evaluation of spinal vascular malformations using the 3 types of postprocessing images. Diagnostic performance was analyzed using receiver operating characteristic analysis, while reader or image differences were analyzed using the Wilcoxon signed-rank test or the Kruskal-Wallis rank sum test. Forty-nine patients (groups A and B: 22 and 27 patients; mean ages, 44.0 [SD, 14.3] years and 44.6 [SD,15.2] years; 13 and 16 men) were evaluated. Junior physicians showed lower diagnostic accuracy and sensitivity using multiplanar reconstruction CTA (85.7% and 77.3%) than senior physicians (93.9% and 90.9%, 98% and 95.5%). Short-term trained juniors achieved SSBBF-CTA image accuracy similar to that of experienced physicians (P > .05). In terms of the visualization and localization of spinal vascular malformation lesions (nidus/fistula, feeding artery, and drainage vein), both multiplanar reconstruction and SSBBF-CTA outperformed routine bone subtraction CTA (P = .000). Compared with multiplanar reconstruction, SSBBF-CTA allowed less experienced physicians to achieve superior diagnostic capabilities (comparable with those of experienced radiologists) more rapidly (P < .05). The SSBBF-CTA technique exhibited excellent reproducibility and enabled accurate pretreatment diagnosis and assessment of spinal vascular malformations with high diagnostic efficiency, particularly for junior radiologists.

Knowledge, attitudes and practices regarding spinal vascular malformations among doctors in China: a cross-sectional study

ObjectiveKnowledge, attitude and practice (KAP) models are essential tools for assessing healthcare professionals’ understanding, beliefs and behaviours towards specific health issues. This study aimed to explore the KAP of Chinese...

Clinical outcomes and prognostic factors in the surgical treatment of spinal dural arteriovenous fistulas: a retrospective study of 118 patients

Spinal dural arteriovenous fistulas (SDAVFs) are the most common type of spinal vascular malformations (AVMs), constituting approximately 70% of all spinal AVMs. The impact of various clinical and radiologic features on the outcomes in patients with SDAVFs is still controversial. The purpose of the study is to investigate the clinical outcomes and prognostic factors in patients with surgically treated SDAVFs in a single center. A retrospective review was performed for all patients with SDAVFs from January 2013 to September 2021 who underwent surgery at our institution. Medical records and pre- and postoperative imaging data were analyzed. Neurological function status was evaluated by modified Aminoff-Logue Scale (mALS). Student’s t-test, Wilcoxon rank sum test, χ2 test and logistic regression were used to find significant prognostic factors. P values < 0.05 were considered significant. One hundred and eighteen patients were ultimately included in the study. By comparing preoperative and postoperative mALS, 69 (58.5%) patients experienced improvement, and 49 (41.5%) patients showed no improvement (worse or unchanged). Wilcoxon rank sum test showed that there was a difference between the improvement group and the no improvement group in preoperative mALS Micturition score and preoperative mALS Defecation score. The logistic regression revealed that preoperative mALS Gait score was associated with clinical improvement after surgery in patients with SDAVFs. Surgical treatment of SDAVFs is a safe and effective procedure and can lead to symptom improvement or stabilization in most patients. Preoperative neurological function status was the only factor associated with clinical prognosis.

Malignancy-related Hypercalcemia Caused by Metameric Cutaneous Metastasis of Parathyroid Hormone-related Protein-producing Bladder Carcinoma with Squamous Cell Differentiation: An Autopsy Case of Cobb Syndrome.

A 74-year-old woman was admitted with hypercalcemia and prolonged disturbance of consciousness. The left buttock to the anterior aspect of the left thigh was swollen and erythematous, with a collection of 1.0-cm large, firm, elastic nodules distributed in a zosteriform pattern in the L1-L4 region. Based on autopsy findings, a very rare case of Cobb syndrome was diagnosed due to a spinal vascular malformation at the Th12-L4 level and L5 vertebral hemangioma. Cobb syndrome-associated cutaneous metastasis extending along the same metamere was complicated by immunohistochemically proven parathyroid hormone-related protein-producing advanced bladder carcinoma in this case.

Surgical evolution in spinal dural arteriovenous fistula treatment-a 7 years monocentric experience.

Accounting for 70% of all spinal vascular malformations, spinal dural arteriovenous fistulas (SDAVF) are the most common type of malformation. Interruption of the fistulous arterialized vein point is the goal of surgical treatment. The aim of the study was to compare open surgery (laminectomy) versus minimal invasive surgery (MIS) in SDAVF treatment. Between March 2013 and March 2020, we retrospectively collected 21 consecutive adult patients with SDAVF. Since March 2017, MIS has been routinely used for surgical treatment. Pre- and post-operative clinical evaluations used Aminoff-Logue score (ALS). Complication rate was noted. Post-operative occlusion of the malformation was confirmed by digital subtraction angiography (DSA) in all patients. MIS was compared to open surgery in terms of efficacy and complications with statistical evaluation. Standard laminectomy was performed in 12 patients and MIS technique in 9 patients. No difference was noted on pre-operative parameters. ALS and MRI signs of myelopathy were improved in all cases except for 1 patient in each group. All SDAVFs were excluded based on post-operative DSA. Significant differences were noted between the 2 groups in terms of perioperative blood loss (p<0.001), post-operative pain visual analog scale values (p<0.001), and first time out of bed (p<0.001). Wrong level surgery occurred in one patient in each group; patients were re-operated using the same technique. No infection or cerebrospinal fluid (CSF) leak was noted. In our experience, MIS is a safe alternative to open laminectomy for SDAVF treatment. MIS contributes to patient comfort and minimizes blood loss without increasing complication rate.

Extradural lumbar arteriovenous fistula imitating a synovial cyst: illustrative case.

Spinal dural arteriovenous fistula is the most common spinal vascular malformation. It usually presents with neurological deficits due to venous congestive myelopathy. Sometimes, however, the symptoms are radicular and can imitate those of a disc hernia or synovial cyst. In this illustrated case, a 34-year-old male patient presented with radicular pain due to nerve root compression. In the magnetic resonance imaging (MRI) examination of the spine, a synovial cyst was suspected, so the patient underwent no further examination before surgery. Intraoperatively, the authors were surprised to see a vascular malformation, which was shown to be an extradural arteriovenous fistula according to the histopathological examination. In atypical MRI findings of the spine, vascular malformations should be considered. In cases with a spinal dural arteriovenous fistula, the thrombosis risk can be high and lead to a need for prolonged anticoagulation.

Open surgical ligation of a thoracic spinal epidural arteriovenous fistula causing thoracic myelopathy: illustrative case.

Spinal epidural arteriovenous fistulas (eAVFs) are rare spinal vascular malformations characterized by an abnormal connection from the paraspinal and paravertebral system to the epidural venous plexus. This contrasts with the more frequently seen spinal dural AVF, where the fistula is entirely intradural. Although endovascular repair is commonly performed for spinal eAVF, few cases require open surgical ligation. The authors present a case of a 74-year-old male with progressive thoracic myelopathy secondary to a spinal eAVF. Thoracic magnetic resonance imaging (MRI) showed intramedullary T2 signal hyperintensity from T8 to T12. Spinal angiography revealed a primary arterial supply from the right T11 segmental artery and minor supply from the left T11 branches with drainage into the ventral epidural space. The patient underwent T11-12 laminectomy and complete right T11-12 facetectomy for ligation of the fistula with T11-L1 fusion. A postoperative spinal angiogram showed resolution of the fistula. Postoperatively, the patient's myelopathy improved, and MRI showed a decrease in T2 cord intensity. Spinal eAVFs are rare lesions that differ from the more commonly seen intradural dural AVF in that the abnormal connection is in the epidural space, and they are often associated with a dilated epidural venous pouch. Treatment involves endovascular, open surgical, or combined approaches.

Spontaneous obliteration of a spinal perimedullary fistula.

We herewith report a young patient who had an incidental spinal vascular malformation of the cervicomedullary junction discovered during a work-up for anosmia. Angiography demonstrated a perimedullary spinal arteriovenous fistula with supply from lateral spinal arteries arising from bilateral V3 level segmental arteries. It was decided to manage the patient conservatively with magnetic resonance imaging monitored biannually. On a recent follow-up magnetic resonance, nearly 10 years later, we noted a subtle change in caliber and imaging characteristics at the posterior margin of the cervical medullary junction. Repeat digital-subtraction angiography showed no evidence of early venous filling from the previously involved branches. Microcatheter exploration of the right lateral spinal artery confirmed spontaneous occlusion of the spinal perimedullary arteriovenous fistula, without any persistent shunting. Spontaneous resolution of a spinal vascular malformation is rare; this case demonstrates the dynamic nature of shunting vascular malformations and that spontaneous obliteration of arteriovenous shunts is possible.

Spinal vascular malformation in a 12 years old child. Diagnostic challenges and management strategies adopting a multidisciplinary approach.

Background: Spinal vascular malformations (SVM) are a complex entity and rarely noted in children. They are commonly seen in the elderly with a predilection for the thoraco-lumbar region. The incidence varies from 3-11% and varies across all age groups. The presenting features in the initial stages are paraplegia, abnormal gait, paraesthesia, radicular pain and diffuse sensory symptoms. If not detected early they can lead to further spinal cord compression and cause urinary retention, bladder, bowel incontinence or erectile dysfunction. Despite the advances in diagnosis and treatment, the complexity of the pathology makes treatment challenging in a child.&#x0D; Case presentation: We present a 12 years old female child who presented with lower limb weakness, diminished reflexes and urinary retention. Nerve conduction velocity was suggestive of motor axonal neuropathy. Magnetic resonance imaging of spine was suggestive of paraspinal vascular malformation and has recovered after surgical intervention and embolization.&#x0D; Conclusion: Spinal vascular malformation is extremely rare in children with very few reported cases. A multi-disciplinary approach beginning with a detailed history taking and clinical examination is invaluable in such complicated cases to deal with the various aspects of a complex pathology.

Review of spinal dural arteriovenous fistulas: Challenges, diagnostics, management, and pathophysiology.

Spinal dural arteriovenous fistulae are rare, spinal vascular malformations that commonly present with progressive myelopathy in a specific demographic and are treatable with surgery (preferred) and/or endovascular embolization. PubMed and Google Scholar were searched with terms including but not limited to "spinal dural arteriovenous fistula", "imaging", "management" "surgery vs embolization", "outcomes", "pathogenesis" to find relevant studies, including emerging research. The purpose of this literature review is to highlight presentation, imaging characteristics, management strategies, pathophysiology, and future directions for these rare but distinct entities.

Recurrence Rates Following Treatment of Spinal Vascular Malformations: A Systematic Review and Meta-Analysis

Spinal vascular malformations (SVMs), including arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs), are a varied group of vascular lesions that can be subclassified according to localization, vascular structure, and hemodynamics. Early intervention is necessary to halt progression of disease and minimize irreversible dysfunction. We sought to characterize initial treatment success and recurrence rates following interventional treatment of various types of SVMs. A systematic review and meta-analysis were performed following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. SVMs were categorized into 4 groups: dural AVFs, perimedullary AVFs, intramedullary AVMs, and extradural-intradural AVMs (e.g., epidural, paraspinal). Initial occlusion, recurrence, and complication rates were compared using random-effects analysis. There were 112 manuscripts included, with a total of 5626 patients with SVM. For treatment, 2735 patients underwent endovascular embolization, 2854 underwent surgical resection, and 37 underwent stereotactic radiosurgery. The initial treatment success and overall recurrence rates following surgical resection of all SVMs were 89.5% (95% CI: 80.5%-98.5%) and 2.3% (95% CI: 0.9%-3.7%), respectively. Those rates following endovascular embolization were 55.9% (95% CI: 30.3%-81.5%) and 27.7% (95% CI: 11.2%-44.2%), respectively. Higher rates of initial treatment success and lower rates of recurrence with surgery were observed in all subtypes compared to embolization. Overall complication rates were higher after embolization for each of the SVM categories. Surgical resection of SVMs provided higher rates of initial complete occlusion and lower rates of recurrence than endovascular techniques. Attaining technical success through obliteration must still be weighed against clinical impact and natural history of the specific vascular malformation.

Transvenous embolization of conus spinal arteriovenous malformation: illustrative case

Spinal arteriovenous malformations (AVMs) are the most challenging spinal vascular malformations. Endovascular transarterial embolization of spinal AVM has been well described. However, transvenous embolization has not been previously reported. In this case report, the authors describe transvenous embolization of a recurrent intramedullary/conus spinal AVM that was previously deemed unamenable to endovascular therapy. A 30-year-old female presented with debilitating lumbar back pain and was found to have an intramedullary spinal AVM at the level of the conus medullaris. Initially, the AVM was treated with endovascular arterial embolization and subsequently with microsurgical resection. However, on follow-up angiography, the AVM had recurred. Further arterial embolization was deemed not possible because of anatomical challenges. After careful consideration, endovascular transvenous embolization was performed with successful occlusion of the AVM nidus. Transvenous embolization is an alternative route for endovascular treatment of spinal AVMs that have an accessible single draining vein.

Coexisting filum terminale arteriovenous fistula and filum terminale lipoma treated with single-stage surgery: illustrative case

Both filum terminale arteriovenous fistulas (FTAVFs) and filum terminale lipomas (FTLs) are rare. Because of this rarity, there is a paucity of data regarding concomitant FTAVF and FTL, and the optimal treatment remains to be defined. The authors describe a patient with coexisting FTAVF and FTL treated with single-stage surgery. A man in his 70s was referred to the authors' department because of a suspected spinal vascular malformation seen on magnetic resonance imaging that was performed to investigate lower limb weakness, intermittent claudication, and urinary incontinence. Previous imaging had shown a terminal lipoma with an internal flow void. Computed tomography angiography and digital subtraction angiography revealed an FTAVF, the feeder being a lateral sacral artery. The patient was treated surgically with curative intent. The FTL and tethered cord that had been identified by imaging were treated in the same procedure. Postoperative digital subtraction angiography showed absence of the abnormal vessels. The patient was discharged home on the 19th postoperative day. When considering treatment, it is important to determine whether symptoms are attributable mainly to FTL, tethered cord, or FTAVF. One-stage treatment is useful because it eliminates both the FTAVF and the factors that led to its development.

Ruptured isolated spinal artery aneurysms: Case series of five patients and a review of the literature on management strategies.

Aneurysms of the spinal arteries are rare entities, the majority of which are associated with other vascular lesions, such as spinal vascular malformations. Isolated spinal artery aneurysms (SAAs) are even less frequently encountered, and their incidence is largely unknown, as the literature is limited to case reports and small series. The optimal management strategy for SAAs is not well defined. To review the institutional experience of five patients with isolated SAAs. Five cases of isolated SAAs were identified at our institution, including two cases with multiple simultaneous SAAs. Clinical presentation, imaging, and management strategies for each case were reviewed. A literature review of all SAAs reported between 1950 and 2020 was performed. A total of five patients with eight isolated SAAs presented to our institution: one aneurysm was lumbar in location, while the remaining seven were thoracic. Two patients were treated with glue embolization followed by laminectomy for hematoma evacuation; one was treated with only surgery; and the other two, which both had multiple lesions, were managed conservatively with interval complete regression of their aneurysms. All five patients had good neurological outcomes. Literature review found 124 patients with at least 137 isolated SAAs and revealed treatment strategies including conservative management, glue or coil embolization, muslin wrapping, and surgical resection or clipping. Multiple management strategies exist for SAAs, and clinical consideration of patient presentation and lesion morphology determine appropriate strategy. Our case series demonstrates three of these treatment paradigms.

Vascular Malformations of the Spinal Cord in Children.

Vascular malformation of the spinal cord in children is a rare and complicated disease spectrum. We will start from the basic spinal cord vascular anatomy and the controversial classification of this kind of disease. Then, we will elaborate the clinical manifestations, diagnostic imaging and treatment of pediatric spinal vascular malformations based on the practical experience of our center and from literature.

Von Hippel-Lindau disease with intracranial hemorrhage due to arteriovenous anastomosis via multiple spinal hemangioblastomas: illustrative case.

Some spinal hemangioblastomas (HBLs) resemble spinal vascular malformations. Intracranial subarachnoid hemorrhage (SAH) secondary to spinal HBL has rarely been reported. A 67-year-old man with a prolonged von Hippel-Lindau disease (VHL) history presented with sudden headache and vomiting. Cranial and cervical computed tomography (CT) revealed severe infratentorial, supratentorial, and cervical SAH. Cranial CT angiography and magnetic resonance imaging revealed a mismatch in hemorrhage and intracranial tumor localization, with no vascular lesions that could lead to intracranial SAH. Cervical CT angiography revealed abnormal blood vessels originating from 5 spinal tumors suspected to be HBLs. We considered that the SAH was caused by venous reflex from vascular malformation-like spinal HBLs. Transarterial embolization (TAE) of the feeding artery of HBLs was performed to improve symptoms and reduce rebleeding risk. Nine months after TAE, angiography showed no venous reflux into the intracranial space. Ten months later, the authors excised the T1-2 tumor because the patient complained of progressive paralysis of the right upper extremity. In HBL with prolonged VHL, intracranial hemorrhage due to venous regurgitation via a mimicked vascular malformation may occur. Reducing venous reflux with TAE may improve symptoms and prevent rebleeding.

Single Centre Initial Experience with the Scepter Mini Balloon Microcatheter.

The use of balloon microcatheters in interventional neuroradiology is well documented. However, their use is sometimes limited by the small diameter and excessive tortuosity of the vasculature. The Scepter Mini dual-lumen balloon microcatheter (SMBM) (Microvention, Aliso Viejo, CA) has been designed to address these challenges by decreasing the distal catheter profile, allowing distal access to the target vessel. This is a single-centre retrospective analysis of the initial cases performed using the Scepter Mini balloon microcatheter. The targeted conditions were vascular malformations. Patient clinical data, angiographic features of the vascular abnormalities and operation reports were reviewed and the procedural parameters, radiation doses, occlusion rates and complications were assessed. A total of 15 SMBM were used in 11 cases. In all cases the procedure performed was balloon inflation and antegrade delivery of precipitating hydrophobic injectable liquid (PHIL) (Microvention, Aliso Viejo, CA) for embolisation of a targeted feeding vessel and cranial and spinal vascular malformations. Successful feeding vessel distal access and antegrade liquid embolisation was achieved in 100% of the cases. One of the 11 cases was an emergency procedure. One procedural target vessel rupture, likely due to overinflation, and three minor post-procedure complications were observed. The SMBM represents a significant advance in the treatment of cerebrospinal vascular malformations, allowing balloon catheter access into tortuous and small calibre vessels.