A New Perspective On Arterioectatic Spinal Angiopathy with a Reversible Pattern: Cause or Consequence?

OBJECTIVE: In 2022, arterioectatic spinal angiopathy (AESA) of childhood was reported as a fatal, progressive, multi-segment myelopathy associated with a unique form of non-inflammatory spinal angiopathy involving diffuse dilatation of the anterior spinal artery and cord congestion in children. In this study, we present four more cases of AESA, using early and long-term conventional imaging and flat detector computed tomography angiography (FDCTA) imaging to assess the probability of disease regression and prevent unnecessary interventions. METHODS: We retrospectively reviewed the clinical and radiological findings of four patients with AESA seen in two neuroradiology departments between 2014 and 2023. RESULTS: The study included three boys and one girl. Two of the boys were siblings. Although the clinical and radiological presentation in the early stages of the clinical course overlapped the definition of AESA, the clinical course was more benign in three of the cases. The clinical courses of the two siblings with monosegmental cord involvement and largely reversible radiological findings suggest that some of the features in the initial definition of the disease cannot be standardized for all patients. The siblings had a mutation of the NDUFS gene, which is involved in mitochondrial function and clinical-radiological reversibility in these patients. CONCLUSION: Many mitochondrial diseases, such as this NDUFS mutation, present with myelopathy, and mitochondrial diseases can sometimes show spontaneous recovery. It is crucial to identify other genetic mutations or environmental factors that trigger the accompanying vascular ectatic findings in AESA in larger multicenter studies to prevent its potential lethal course and possible unnecessary surgical-endovascular interventions.

Intracranial subarachnoid hemorrhage as part of spinal arteriovenous metameric syndrome.

Spinal Arteriovenous Metameric Syndrome is a rare and complex nonhereditary genetic vascular disorder, affecting multiple layers of tissues at the same metamere, including the spinal cord. We present a case of a 20-year-old man who presented to the emergency department with sudden headache and transient loss of consciousness. Cranial computed tomography scan revealed subarachnoid hemorrhage predominantly in the cerebellar cisterns, fourth ventricle, extending to the basal cisterns. Cerebral angiography showed no abnormalities. Cervical angiographic acquisitions demonstrated a spinal metameric arteriovenous malformation (AVM) at the C3 and C4 levels. Cervical magnetic resonance imaging also confirmed the metameric AVM, revealing both intradural intramedullary and extra-dural vascular lesions in the vertebrae and adjacent soft tissues. The patient was referred for endovascular treatment. Although quite rare, the association between cervical spinal arteriovenous shunt diseases and intracranial hemorrhage has been reported. The bleeding in this case may be attributed to venous reflux into intracranial veins.

Localization of spinal dural arteriovenous fistulas from the spatial relationships of perimedullary vessels on standard MRI.

OBJECTIVE: The goal in this study was to explore the spatial relationship of perimedullary vessels visualized on MRI to localize the side and the site of spinal dural arteriovenous fistula (SDAVF). METHODS: A retrospective analysis of 30 consecutive patients diagnosed with SDAVF on MRI was undertaken. Two experienced reviewers blinded to all reports and angiographic images analyzed T2-weighted as well as postcontrast T1-weighted sequences. A focal prominent zone of perimedullary vessels with lateralization to one side in the thecal space was evaluated to locate the side and the site of the fistula. Spinal digital subtraction angiography served as the gold standard technique. RESULTS: Good interrater agreement (κ = 0.77) was shown for the diagnosis of SDAVF with perimedullary vessels on T2-weighted MRI. Flow voids on T2-weighted MRI demonstrated a sensitivity of 1.0 (95% CI 1.0-1.0) and an accuracy of 0.87 (95% CI 0.79-0.95) to identify the presence of fistula. The flow voids on T2-weighted MRI also demonstrated 0.88 (95% CI 0.71-1.03) sensitivity and 0.81 (95% CI 0.70-0.92) accuracy to identify the side of SDAVF. Furthermore, flow voids on T2-weighted MRI showed 0.87 (95% CI 0.71-1.03) sensitivity and 0.87 (95% CI 0.79-0.95) accuracy to identify the site of SDAVF within 3 vertebral levels above or below the actual site. Area under the receiver operating characteristic curve demonstrated significant results (0.87 [95% CI 0.73-1.0]; p < 0.001) for flow voids on T2-weighted MRI to identify the site of shunts within 3 vertebral levels in the cranial or caudal direction. CONCLUSIONS: Spatial distribution of perimedullary vessels observed on standard MRI show promise to locate the side and the site of fistula in patients with SDAVF.

Spinal Dural Arteriovenous Fistulas: A Retrospective Analysis of Prognostic Factors and Long-Term Clinical Outcomes in the Light of the Recent Diagnostic and Technical Refinements.

Spinal dural arteriovenous fistulas (dAVFs) are abnormal connections between the meningeal branches of segmental arteries and a radiculomedullary veins that result in a progressive myelopathy thanks to perimedullary coronal venous plexus congestion. Usually, dAVFs show nonspecific symptoms, thus leading to late clinical suspicion and a difficult MRI diagnosis.Several authors have tried to identify prognostic factors before treatment, but published studies results are often inconsistent and sometimes contradictory.In this study, we reviewed our recent experience of 30 dAVF patients where we collected all demographic, clinical and angioarchitectural features as well as radiological and treatment-related characteristics. The thoracic spine was the most common location, constituting 53.3% of cases, followed by the lumbar roots, comprising 30%. About 83% of patients showed motor deficits, urinary disturbances were present in 70%, and bowel symptoms in 50%.We treated 86.7% of patients with microsurgery and 13.3% with endovascular occlusion with a mean interval between clinical onset and intervention of 10.8 ± 14.2 months.A significant clinical improvement was observed at follow-up in 80% of patients, with a significant reduction in mean G-score, U-score and F-score at a mean follow-up of 105.89 ± 191.9 months.However, none among the principal demographic, clinical and radiological characteristics showed significant prognostic value to the clinical improvement observed at follow-up.

Autoimmune glial fibrillary acidic protein astrocytopathy complicated with low flow perimedullary arteriovenous fistula: a case report.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy and low-flow perimedullary arteriovenous fistulas (PMAVFs) may cause longitudinal widespread myelopathy. We report a middle-aged male patient with autoimmune GFAP astrocytopathy complicated with low flow PMAVFs disease, presenting with lower extremity weakness and dysuria. Magnetic resonance imaging (MRI) of the spinal cord revealed a significant longitudinal extent of T2 high signal from T11 to L1, with the lesion located proximal to the vascular territory supplied by the anterior spinal artery. Multiple patchy abnormal signals were seen adjacent to the anterior and posterior horns of the lateral ventricles bilaterally and at the centers of the semi-ovals on MRI of the cranial brain, with iso signal in T1Flair, the high signal in T2WI, and no high signal seen in Diffusion Weighted Imaging (DWI). Subsequently, the presence of anti-GFAP antibodies was detected in the cerebrospinal fluid (CSF), and the diagnosis of autoimmune GFAP astrocytopathy in conjunction with low-flow PMAVFs was confirmed through spinal digital subtraction angiography (DSA). This case report aims to increase neurologists' awareness of this disease and avoid missed or misdiagnosed cases that may lead to delayed treatment.

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.

De Novo Radicular Arteriovenous Fistula After Treatment of Spinal Arteriovenous Fistula: A Case Report and Literature Review.

De novo spinal dural arteriovenous fistulas (AVFs) have been reported as metachronous AVFs However, metachronous spinal AVFs are extremely rare, and their pathogenesis remains uncertain. We report a case of de novo radicular AVF (RAVF) following treatment for spinal AVF at the craniocervical junction (CCJ). We also reviewed the literature and discussed the pathogenesis of metachronous spinal AVF. A 64-year-old male patient diagnosed with spinal AVF at the CCJ supplied from the right C1 segmental artery was treated with Onyx-18 (eV3 Inc, CA, USA) trans-arterial embolization, resulting in partial occlusion. Angiography showed a slight residual shunt two weeks after the embolization without another shunt lesion. A five-year follow-up spinal angiography showed de novo RAVF at the C4 level and complete occlusion of the first AVF. The second AVF was not treated because it was asymptomatic, and the patient remained asymptomatic. De novo RAVF was found to develop five years after the embolization of a CCJ-spinal AVF in a patient. This is the first case of de novo RAVF post-treatment of a spinal AVF. This case demonstrated that RAVF could develop as an acquired disease.

Spontaneous regression of extradural high-flow vascular malformation in spinal arteriovenous metameric syndrome (SAMS): A unique case report.

Background: Spinal arteriovenous metameric syndrome (SAMS) is a rare nonhereditary genetic vascular disorder, involving multiple layers of tissues in the same metameric level. Spontaneous regression of SAMS has never been reported in the medical literature. Case Description: A 42-year-old woman presented with intermittent low back pain for 6 months. Magnetic resonance imaging of the thoracolumbar spine incidentally found clusters of spinal vascular malformations involving spinal cord, vertebral bodies, epidural space, and paraspinal muscles. There was no sign of venous congestion. Magnetic resonance angiography and spinal angiography revealed intradural spinal cord arteriovenous malformation (SCAVM) at the level of T10-11 and extradural high-flow osseous arteriovenous fistula. Due to asymptomatic SAMS and a high risk of anterior spinal arterial compromise during treatment, conservative treatment was considered in our patient. Spinal angiography obtained 8 years after initial angiography demonstrated significant regression of extradural component of SAMS and stable intradural SCAVM. Conclusion: We describe a unique case of SAMS with spontaneous regression of extradural component during a long-term observation period.

Surgical Management of a Spinal Dural Arteriovenous Fistula.

Spinal dural arteriovenous fistulas (SDAVFs) may have subtle clinical presentations and are often misdiagnosed. Clinical status gradually deteriorates following symptom onset making prompt identification and management essential. Here we present a case of a 67-year-old patient with rapidly progressing motor and sensory deficits to eventual right hemiplegia. Following imaging and surgical intervention, a thoracic SDAVF was identified and resected. This case report highlights a unique SDAVF with a stroke-like presentation. For patients with such presentation, without a clear source of intracranial pathology, spinal causes such as SDAVF could be considered.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Spinal Dural Arteriovenous Fistula in a Young Male Associated with Craniospinal Leptomeningeal Spread of a Treated High-Grade Glioma.

Spinal dural arteriovenous fistulae (SDAVF) are most commonly idiopathic in origin but may occasionally be seen secondary to surgery, trauma, or inflammation. We report a case of 27-year-old male who came with features of a myelopathy. He was found to have an SDAVF associated with leptomeningeal spread (LMS) of a previously treated high-grade cerebral glioma. Hemorrhagic presentation of gliomas, as in this case, is due to upregulation of vascular endothelial growth factor, which has also been postulated to play a role in the development of SDAVFs. This may suggest a possible mechanism of induction of secondary SDAVFs associated with such tumors. While the coexistence of intracranial neoplasms with vascular malformations has been reported previously, this is the first case report of LMS of a high-grade glioma associated with an SDAVF.

Spinal epidural arteriovenous fistula in 3 cats.

Three young adult cats with intermittent spinal hyperesthesia and paraparesis and diagnosed with spinal epidural arteriovenous fistula are described. In all 3 cases, magnetic resonance imaging (MRI) showed focal dilatation of the veins in the epidural space of the thoracic spinal cord, whereas computed tomography angiography (CTA) showed dilatation and enhancement from the intercostal vein to the azygos vein at the same site in the arterial phase. Dorsal laminectomy and occlusion of the interarcuate branches running across the dorsal aspect of the spinal cord were performed in all 3 cats to decompress the spinal cord, which resulted in a remission of clinical signs and no recurrence during 14 to 40 months of follow-up after surgery in all cases.

Recognition of the variant type of spinal dural arteriovenous fistula: a rare but important consideration.

OBJECTIVE: Spinal dural arteriovenous fistulas (SDAVFs) typically represent abnormal shunts between a radiculomeningeal artery and radicular vein, with the point of fistulization classically directly underneath the pedicle of the vertebral body, at the dural sleeve of the nerve root. However, SDAVFs can also develop in atypical locations or have more than one arterial feeder, which is a variant of SDAVF. The aim of this study was to describe the incidence and multidisciplinary treatment of variant SDAVFs in a single-center case series. METHODS: Following institutional review board approval, the authors retrospectively analyzed their prospectively maintained database of patients with SDAVFs who presented between 2008 and 2020. For all patients, spinal digital subtraction angiograms were reviewed and variant SDAVFs were identified. Variant types of SDAVFs were defined as cases in which the fistulous point was not located underneath the pedicle. Patient demographics, angiographic features, clinical outcomes, and treatment modalities were assessed. RESULTS: Of 59 patients with SDAVFs treated at the authors' institution, 4 patients (6.8%) were identified as having a variant location of the shunt zone, pinpointed on the dura mater at the intervertebral level, further posteriorly within the spinal canal. In 3 cases (75%), a so-called bimetameric arterial supply was demonstrated. CONCLUSIONS: Recognition of the variant type of SDAVF is crucial for management, as correct localization of the fistulous point and bimetameric supply are critical for successful surgical disconnection, preventing delay in achieving definitive treatment.

Arteriovenous fistulas in the craniocervical junction region: With vs. without spinal arterial feeders.

Objective: Arteriovenous fistulas (AVFs) in the craniocervical junction (CCJ) region are a rare occurrence with special clinical manifestations. This study retrospectively reviewed patients with CCJ AVFs treated at our neurosurgical center, aiming to enhance the understanding of CCJ AVFs. Methods: A total of 113 patients with CCJ AVFs treated at our neurosurgical center between January 2013 and December 2020 were enrolled. They were grouped as patients with CCJ AVFs with spinal arterial feeders (n = 20) and patients with CCJ AVF without spinal arterial feeders (n = 93). Clinical presentation, angiographic characteristics, intraoperative findings, and treatment outcomes were analyzed. Results: The patients' median age was 55 years (IQR 47.5-62 years). The proportion of males in the group without spinal arterial feeders was significantly higher (p = 0.001). Subarachnoid hemorrhage (SAH) was the most common clinical presentation, especially in the group with spinal arterial feeders (p < 0.001). There were significant differences in AVF type, fistula location, and direction of the venous drainage between the two groups (p < 0.001). Intervention embolization combined with microsurgery was more common in treating AVFs with spinal arterial feeders (p = 0.006). Spinal arterial feeders did not affect the outcome (p = 0.275). Conclusions: SAH was the most common presentation of CCJ AVFs in this study. Microsurgery and interventional embolization were optional treatment strategies. The angioarchitecture of CCJ AVFs was essential for selecting treatment strategies.

Rapid Progression to Complete Paraplegia After Electroacupuncture in a Patient With Spinal Dural Arteriovenous Fistula: A Case Report.

Spinal dural arteriovenous fistula (SDAVF) usually has an insidious clinical course, but 5-15% of the cases have acute exacerbations. In some cases, there is an abrupt progression to paraplegia following an epidural injection or anesthesia. Electroacupuncture is a form of acupuncture that applies a small electrical current to needles inserted at specific points in the body. It is widely used for its analgesic effect on back pain. In this study, we report a rare case of SDAVF in which the symptoms of a patient worsened rapidly to complete paraplegia within a few hours after applying electroacupuncture to his back. A 49-year-old man had rapid progression to complete paraplegia within a few hours of electroacupuncture on his back. MRI showed SDAVF and worsening of cord signal change. An emergency operation was performed to ligate the SDAVF. The patient was able to walk 1 month post-operatively. Most of the neurological deficits had disappeared by 1 year post-operatively, with normalization of MRI. Our case emphasizes that SDAVF patients should be careful when exposed to any circumstances that might affect the circulation around the dural arteriovenous fistula, such as electroacupuncture. Patients should also be warned in advance about the possibility of rapid exacerbation of neurological symptoms. Regardless of the severity of the neurological symptoms, immediate treatment is essential for recovery and a better outcome.

Hirayama-like disease in the thoracic spine.

Hirayama disease is a cervical flexion myelopathy that typically causes upper extremity weakness in young male patients. We present two male patients (age 15 and 29) with MRI findings of thoracic ligamentous laxity similar in appearance to Hirayama disease. However, patients presented with atypical symptoms, specifically back pain and paresthesia of the upper and/or lower extremities, likely correlating to the abnormal thoracic spinal levels involved. Flexion/extension MRI sequences demonstrated the forward displacement of the dorsal dura and compression the thoracic cord with prominence of the posterior epidural space and venous plexus. Follow-up MRAs were negative for a spinal vascular malformation. Patients were managed conservatively with no surgical intervention. Clinical history, thoracic MRI, and follow-up flexion and angiographic imaging sequences may help confirm a diagnosis of Hirayama-like thoracic ligamentous laxity.

Current and novel non-invasive imaging modalities in vascular neurosurgical practice.

Radiological investigations are a powerful tool in the assessment of patients with intracranial vascular anomalies. 'Visual' assessment of neurovascular lesions is central to their diagnosis, monitoring, prognostication and management. Computed tomography and magnetic resonance imaging are the two principal non-invasive imaging modalities used in clinical practice for the assessment of the cerebral vasculature, but these techniques continue to evolve, enabling clinicians to gain greater insights into neurovascular pathology and pathophysiology. This review outlines both established and novel imaging modalities used in modern neurovascular practice and their clinical applications.

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

OBJECTIVE: Vascular 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. METHODS: The 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. RESULTS: Of 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. CONCLUSIONS: This 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.