Intramedullary Arteriovenous Malformations Research Articles

Coil Embolization of a Ruptured Anterior Spinal Artery Aneurysm Associated with Spinal Cord Arteriovenous Malformation.

The present report describes a patient with spinal cord arteriovenous malformation (AVM) and an associated anterior spinal artery aneurysm presenting with subarachnoid hemorrhage. Diagnostic spinal angiography revealed an intramedullary AVM, located at the T10-T11 level, and a prenidal saccular aneurysm at the junction of the radiculomedullary artery and the anterior spinal axis, fed by the right T8 segmental artery. The patient underwent successful selective coil embolization of the aneurysm. Follow-up angiography 3 months postoperatively showed no recurrence of the aneurysm.

Spinal dural arteriovenous fistula: a case-based review

Spinal dural arteriovenous fistula is an abnormal connection between radicular arteries and spinal venous plexus. This condition is relatively rare with annual incidence of five-ten cases per million. We present a case of a 62-year-old male with complaints of tingling in feet since six months and weakness in legs since 1 hour. Lower limb weakness progressed over a day to complete paralysis and bladder incontinence. Magnetic resonance imaging (MRI) spine showed a dural hypointensity over D6-D11 with abnormal vessels around spinal cord. Patient underwent spinal angiography which showed an intramedullary arteriovenous malformation causing compression. Arterial embolization was done within 24 hours from symptom onset and he ambulated within a week. AV malformations are a rare cause of compressive myelopathy presenting with acute paraplegia. Advances in endovascular techniques have made it possible to treat them early and prevent irreversible damage.

A Classification Proposal for Spinal Vascular Lesions.

A Classification Proposal for Spinal Vascular Lesions.

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.

Anterior Spinal Cord Ischemia due to an Acute Aortic Intramural Type B Hematoma

Anterior Spinal Cord Ischemia due to an Acute Aortic Intramural Type B Hematoma

Angiographic Evidence of Response to Trametinib Therapy for a Spinal Cord Arteriovenous Malformation

Several studies have implicated RAS and the RAS-related gene family (eg, BRAF, KRAS, and MAPK) in sporadic arteriovenous malformations (AVMs) with evidence that targeted biologic agents (eg, trametinib) may be beneficial for certain patients. Despite similar genetic mutations in brain and spine AVMs, however, no evidence of molecular intervention has proven effective in impacting such cases. We describe a single patient undergoing MEK-inhibitor therapy for a KRAS-positive thoracic wall AVM with an additional spinal intramedullary AVM. Angiography demonstrated significant reduction in the flow to and size of the patient’s intramedullary AVM. Based on this result, formal trials for inoperable and/or therapeutically challenging central nervous system AVMs might be considered.

Spontaneous cerebral SAH by intranidal aneurysm rupture of cervical intramedullary glomus arteriovenous malformation: case report and review of the literature

Spontaneous cerebral SAH by intranidal aneurysm rupture of cervical intramedullary glomus arteriovenous malformation: case report and review of the literature

Microsurgical or endovascular strategy for complete obliteration of spinal arteriovenous shunts in a single-institute 10-year retrospective study

The objective of this retrospective study is to more fully understand the optimal strategy to manage spinal arteriovenous (AV) shunts. This study included a cohort of 35 patients with a diagnosis of spinal AV shunts who were treated over the past 10 years at a single institute. Angiographic diagnosis of intramedullary AV malformations (IM-AVM), perimedullary AV fistulas (PM-AVF), dural AV fistulas (D-AVF), or epidural AV fistulas (ED-AVF) was carefully made, and the microsurgical or endovascular strategy for them was determined at the interdisciplinary meeting consisting of neurospinal surgeons and endovascular specialists. Endovascular surgery was first considered whenever safely possible. Microscopic direct surgery using intraoperative image guidance was considered for cases in which endovascular access was challenging or not safely possible. Combined treatment was another option. The clinical condition was assessed using the modified Rankin scale (mRS). Seventeen of 35 cases were treated with microscopic direct surgery, 13 cases with endovascular surgery, and the remaining five cases with the combination. Complete angiographic obliteration was achieved in 30 of 35 cases (85.7%). Although residual AV shunts was recognized in 3 cases of IM-AVM, 1 case of PM-AVF and 1 case of ED-AVF, no angiographic recurrence was present with an average postoperative follow-up period of 44 months. The average mRS before surgery was 2.37 and significantly improved to 1.94 at the most recent follow-up. Interdisciplinary collaboration between neurospinal surgeons and endovascular specialists should be standard to achieve safe and successful outcomes in treating such rare and difficult spinal disorders.

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

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. 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. 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. 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.

Spinal Cord Tolerance Guided by Stereotactic Radiosurgical Treatment of Intramedullary Arteriovenous Malformations

Spinal cord toxicity resulting in neurological deficits remains the most feared complication of spinal region stereotactic radiosurgery (SRS). In treating vertebral body disease, a radiation dose gradient can typically be established between tumor and spinal cord. This is difficult for intramedullary spinal cord arteriovenous malformations (iSCAVMs), which can be integrated throughout the cord. Here we report outcomes of cord tolerance in treatment of integrated iSCAVMs. A retrospective cohort of 17 consecutive patients with integrated iSCAVMs treated once with frameless robotic surgery, SRS between 2007 and 2018 was accrued. Clinical outcomes, dose prescriptions, and target dose-volume histogram (DVH) parameters were acquired. Volumes of interest (VOIs) comprising the proper AVM and cord were registered on treatment planning software. A cord minus AVM (CmA) structure representing independent spinal cord was generated by Boolean subtraction of the respective proper AVM from the cord. Various dose quantifiers were extracted from DVHs, statistical univariate analysis was performed, and Task Group 101 (TG101) constraints were interpolated to two-fraction regimens with α/β of 2-3 Gray (Gy) using Wolfram Mathematica (Wolfram Research, Champaign, IL). Among the 17 patients analyzed (eight male and nine female), all had CmA volumes smaller than cord, confirming the integrated nature of their iSCAVMs. Nine had a nidus in the C-spine; six in the T-spine; and two in the L-spine or lower. Median age at diagnosis was 23, ranging from 9 to 56 years. Nine patients had no prior intervention, while eight had prior embolization(s) and/or microsurgery. Median follow-up time was 40 months. Ten patients (59%) experienced Grade (G) 0 neurological toxicity post-treatment, seven (41%) experienced G1, and zero experienced G2+. Three patients were prescribed three fractions to 21 or 24 Gy. The rest received two fractions (20-22 Gy). Max dose (Dmax) to cord and CmA ranged from 2278-2874 cGy and 2000-2659 cGy, respectively. All patients had Dmax to cord and CmA greater than TG101 constraints. All patients received minimum dose to hottest 0.03 cc volume (D0.03) to cord greater than constraints; 15 patients (88%), to CmA. All patients received minimum dose to hottest 0.35 cc volume (D0.35) to cord greater than threshold; 13 patients (76%), to CmA. At significant level of 0.05, one-tailed binomial test suggests max monthly G2+ toxicity rate of 0.4% in exceeding tolerances. No neurologic toxicity beyond Grade 1 was experienced by any patient, all of whom received Dmax to spinal cord tissue (both independent and integrated with AVM) beyond recommended constraints. Further, all patients exceeded volumetric constraints to cord. Most exceeded volumetric constraints to CmA, as well. Novel tolerance data from rare iSCAVMs may influence current cord constraints to avoid limiting appropriate treatment of more common vertebral metastases.

Endovascular and microsurgical treatment for spinal arteriovenous malformations. Our experience

Objective – to improve the treatment quality for patients with spinal arteriovenous malformations (AVM).Materials and methods. A retrospective analysis of endovascular and surgical treatment of patients was made. In SO «Scientific-practical Center of endovascular neuroradiology NAMS of Ukraine» since 2005 till 2018 were operated 55 patients (34 (61.8 %) men and 21 (38.2 %) women). The age of patients ranged from 11 to 62 years (middle age is 45.3 years). The classification proposed by Anson and Spetzler in 1992 was used in order to divide patients into experimental groups: type I, arteriovenous fistula (AVF); type II, glomus intramedullary AVM; type III, juvenile AVM; and type IV, perimedullary AVF. Patients with type I were 36 (65.5 %), with ІІ type – 10 (18.2 %), with ІIІ type – 5 (9.0 %), with IV type – 4 (7.3 %). For diagnostics were used MRI and spinal angiography. The Aminoff–Logue scale was used for assessment of the neurological deficiency severity. Early postoperative complications (CSF) leakage, wound infection, early postoperative worsening) were counted.Results. 12 (21.8 %) patients were treated using the microsurgical method and 43 (78.2 %) were treated endovascular. We used endovascular and microsurgical methods to treat patients with I type spinal AVM. Microsurgical intervention for 9 (33 %) patients was performed. Complete disconnection of fistula was achieved in 9 patients. Early postoperative complication (CSF leakage) was observed in 1 (11 %) case. Endovascular treatment was performed for 27 (67 %) patients. Total disconnection was achieved in 16 (59 %) cases, 17 patients (63 %) had early transient neurological worsening. Embolization is the first-line treatment for patients with type II AVM – 8 (80 %) patients. Complete obliteration of AVM was attained in 5 patients (62.5 %), 6 (75 %) patients suffered from early worsening of postoperative neurological symptoms. The microsurgical method was used in case of low risk of spinal cord injury in 2 patients (20 %), AVMs were excluded totally in 2 patients and in 1 patient neurological deterioration was stabilized. All patients with III type AVM were treated endovascular. AVM was obliterated complete in 1 patient (20 %). Three (60 %) patients had transient neurological deterioration. Endovascular method was used to treat 3 (75 %) patients with type IV AVM. Totally disconnection of AVM was achieved in 1 (33 %) patient. Super-selective catheterization of all conductive arteries was not possible. Neurological worsening was observed in 1 (33 %) patient.Conclusions. Angiography is the first-line diagnostic method which permits to find the most effective and safe way (endovascular or/and microsurgical treatment) to disconnect AVM from spinal cord bloodstream. Microsurgical intervention helps to achieve eye-controlled total arteriovenous disconnection. Microsurgical technique usage leads to good neurological results – neurological improvement the following day after surgery in all cases of AVM type I treatment. The structure of AVM determines effectiveness, radicality, method of treatment and the level of feeder artery catheterization. The main goal of embolization to achieve the safe catheterization level of the afferent. Multimodal treatment, which includes endovascular and microsurgical methods, is the most effective in case of AVM II and III types.

Stereotactic Radiotherapy for Intramedullary Spinal Arteriovenous Malformations.

IntroductionPreliminary results of stereotactic radiotherapy (SRT) for spinal arteriovenous malformation (AVM) in five cases are presented. MethodsTwo cases were male and three were female. Their median age was 32 years (range: 23 to 54 years). The spinal intramedullary AVMs were located in the cervical spine in three and in the thoracic spine in two. SRT with 20 Gy in four fractions was delivered to the nidus in every case.ResultsAt the end of the median follow-up period of 5.5 years (range: 3 to 8.5 years), the neurological symptoms and signs were improved or unchanged as compared with before treatment, although a temporary adverse effect developed between a half year and one year after SRT in one case. The nidi were obliterated five and a half years after SRT in one case and three years after SRT in one. In the other three cases, the nidi were unchanged.ConclusionSRT with 20 Gy in four fractions was thought to be a safe treatment, though this study dealt with only a small number of patients with a short-term follow-up period.

Spinal Cord Vascular Disease

The spinal cord is subject to many of the same vascular diseases that involve the brain, but its anatomy and embryology render it susceptible to some syndromes that do not have intracranial counterparts.The embryonic arterial supply to the spinal cord derives from intradural vessels that enter at each spinal level and divide to follow the dorsal and ventral roots.SPINAL CORD ISCHEMIA: The midthoracic levels of the spinal cord are traditionally considered to be the most vulnerable to compromise from hypoperfusion, but more recent evidence suggests that the lower thoracic cord is at greater risk . The actual prevalence of spinal cord infarction is unknown, but is generally cited as representing 1% to 2% of all central neurovascular events and 5% to 8% of all acute myelopathies.Weakness (100%), sensory loss (89%), back pain at onset (82%), and urinary complaints requiring catheterization (75%) were the most common symptoms of cord ischemia at the time of presentation . Weakness most commonly affects both legs. Examination typically reveals flaccid paresis accompanied by diminished superficial and tendon reflexes below the level of the lesion. Preservation of strength and reflexes suggests the rare syndrome of posterior spinal artery territory infarction. Weakness most commonly affects both legs. Examination typically reveals flaccid paresis accompanied by diminished superficial and tendon reflexes below the level of the lesion. Preservation of strength and reflexes suggests the rare syndrome of posterior spinal artery territory infarction. Aortic pathologies with regional hemodynamic compromise are the most common cause of spinal cord infarction, accounting for 30% to 40% of cases. The medical management of spinal cord ischemia is generally supportive and focused on reducing risk for recurrence.SPINAL VASCULAR MALFORMATIONS :Spinal vascular malformations consist of normal-sized to enlarged arteries and enlarged, tortuous veins without an intervening capillary network. A commonly accepted classification system categorizes spinal vascular malformations into four types:Type I—dural arteriovenous fistula (AVF); subtypes IA (single feeding artery) and IB (multiple feeding arteries).Type II—intramedullary glomus-type arteriovenous malformation (AVM).Type III—intramedullary juvenile-type AVM, which is more extensive than a glomus-type AVM, frequently having an extramedullary component and sometimes an extradural component.Type IV—intradural, extramedullary (perimedullary) AVF: subtypes IVA, IVB, and IVC correspond to lesions with progressively increased arteriovenous shunting manifested as increased number, size, and tortuosity of feeding arteries.Spinal vascular malformations, especially dural AVFs, are frequently misdiagnosed. The onset of manifestations can be acute or insidious, and the course may include remissions and relapses. The most common complaints at onset are pain, weakness, and sensory symptoms.

Chapter 16 - Endovascular treatment of spinal arteriovenous malformations

Spinal arteriovenous malformations (AVMs) are a rare but treatable cause of myelopathy and spinal cord hemorrhage. The clinical presentation and ideal treatment of these lesions vary widely, primarily due to differences in anatomic and angioarchitectural features. Six well-defined types of fistula have been defined: (1) intradural dorsal AVM; (2) intramedullary AVM; (3) extradural-intradural AVM; (4) intradural ventral AVF; (5) extradural/epidural AVF; and (6) conus medullaris AVM. Each of these lesions have unique clinical presentations and variable natural histories and endovascular treatment options. The principles of endovascular treatment include: (1) a complete understanding of the angioarchitecture and anatomic features of the lesion, including identification of radiculomedullary arteries; (2) careful spinal angiography; (3) identification of treatment goals prior to the procedure (i.e., complete occlusion versus partial occlusion aimed at altering natural history); (4) selection of proper embolic agent; and (5) careful follow-up in both the immediate postoperative setting and long term.

Chapter 15 - Spinal arteriovenous malformations: surgical management

Spinal arteriovenous malformations (AVMs) are a diverse population of vascular lesions associated with significant long-term morbidity and neurologic impairment. Spinal AVMs can be classified into three categories: intramedullary, extradural-intradural, and conus, depending on their angioarchitecture and anatomy. Extradural-intradural lesions are typically incurable due to their diffuse nature, but patients may benefit from targeted therapy of symptomatic components. Intramedullary and conus AVMs are amenable to safe surgical resection when combined with endovascular embolization and use of a pial dissection technique that spares entry into the neural parenchyma.

Microsurgical management and outcomes of intramedullary spinal arteriovenous malformations: a report of 14 cases

Objective: To investigate the clinical features, microsurgical management and outcomes of intramedullary spinal arteriovenous malformations (AVM). Methods: A series of 14 patients (10 male, 4 female; mean age (39.1±17.2) years ranged from 16 to 72 years) with intramedullary spinal AVM who underwent microsurgical treatment from November 2008 to April 2015 in Department of Neurosurgery at Beijing Tiantan Hospital were retrospectively reviewed. The location of the AVM included cervical (n=6), thoracic (n=3), cervicothoracic (n=2), and conus medullaris (n=3). The patients underwent microsurgery via the posterior myelotomy, indocyanine green fluorescence angiography(ICG)was performed to detect the feeding arteries and draining veins. After the lesions were removed, ICG was performed again to judge whether the AVM was resected completely. Results: Fourteen patients underwent resection of intramedullary spinal AVM in this period, total AVM resection was achieved in 12 patients and subtotal in 2 patients. The amount of bleeding was (178.6±141.0) ml ranged from 100 to 600 ml intraoperatively and no one received blood transfusion. Clinically, 13 patients improved, 1 patient worsened. Twelve patients got functionally improved, 2 patients remained unchanged during the long-term follow-up period ranged from 11 to 84 months, with a mean time of (60.9±20.4) months. Conclusions: Intramedullary spinal AVM is an extremely rare spinal vascular disease. Microsurgical treatment can safely resect the lesions with satisfactory clinical outcomes and reduce spine function deterioration.

Microsurgical Resection of Cervical Spinal Cord Arteriovenous Malformations: Report of 6 Cases

To summarize the experience in microsurgical resection of cervical spinal cord arteriovenous malformation (AVM). Six patients undergoing microsurgical resection of cervical intramedullary AVM in the Third Affiliated Hospital of Sun Yat-sen University, China, between March 2005 and March 2015 were reviewed retrospectively, and their clinical manifestations, imaging data, surgical treatment, and results of long-term follow-up were analyzed. Of the 6 patients who underwent AVM resection, 2 had compact AVMs and 4 had diffuse AVMs. All 6 patients were reexamined with spinal magnetic resonance imaging within 48 hours after surgery, and 1 patient was examined with digital subtraction angiography. The average patient age was 40.7 ± 10.4 years (range, 29-60 years). Three patients had chronic onset, of whom 2 developed sensory disturbances and 1 had muscle weakness. The other 3 patients had acute onset, including 1 with sudden quadriplegia, 1 with idiopathic severe headache and altered consciousness, and 1 with idiopathic neck pain. The average duration of follow-up was 48.5 ± 38.9 months (range, 15-119 months). One patient experienced complete recovery, and the other 5 patients showed improvement. No patient exhibited deterioration. Microsurgical resection of cervical intramedullary AVMs has obtained satisfactory clinical results. Preoperative magnetic resonance angiography, computed tomography angiography, and digital subtraction angiography are useful for evaluating the angioarchitecture, which is key to the success of surgery. Intraoperative indocyanine green fluorescence angiography is an important aid in the surgical treatment of spinal AVMs.

Stereotactic radiosurgery as a feasible treatment for intramedullary spinal arteriovenous malformations: a single-center observation.

Spinal cord intramedullary arteriovenous malformations are rare. For patients suffering from either hemorrhage or myelopathy, surgical or endovascular interventions are indicated. However, complete eradication of the nidus is often difficult because of its intramedullary location and complex angioarchitecture. In this report, we evaluate the feasibility and safety of stereotactic radiosurgery as a treatment modality for intramedullary spinal arteriovenous malformations (AVMs). Between 2010 and 2014, we performed stereotactic radiosurgery to treat four patients with intramedullary AVM and one with spinal arteriovenous metameric syndrome (one woman and four men; age range, 31-66years). Three patients presented with myelopathy, and two suffered hemorrhages. Nidi were located in the cervical (three cases) and thoracic (two cases) spinal cord regions. Based on the angioarchitecture, surgery and endovascular embolization were indicated. When both modalities were deemed hazardous, radiosurgery using CyberKnife™ was offered. Radiation using marginal doses of 18Gy was administered in three fractions. The mean follow-up period was 37.2months (range, 16-62months). After treatment, two of the three patients with myelopathy experienced either improvement or stabilization of their symptoms and one experienced worsening of dysesthesia. In two patients with hemorrhage, symptoms improved in one and remained stable in the other. No further hemorrhagic episodes were evident during follow-up. Follow-up angiograms showed marked shrinkage of the nidus located in the thoracic spinal cord in one case and angiographic stabilization in the others. As a treatment modality for intramedullary AVMs, CyberKnife™ is safe and can be considered when surgery or endovascular therapy is not indicated. To determine optimum radiation doses and protocols for treating spinal AVMs, further studies with more patients and long-term follow-up are required.

Pediatric spinal arteriovenous malformations and fistulas: a single institute’s experience

Pediatric spinal vascular diseases are extremely rare. We aimed to evaluate their clinical characteristics and treatment outcomes in our institute. A total of 10 patient files were retrospectively reviewed, including 3 cases of intramedullary arteriovenous malformations (AVMs), 6 cases of perimedullary arteriovenous fistulas (AVFs), and 1 case of epidural AVF. Clinical features, radiological findings, treatment results, and clinical outcomes were evaluated. The median durations of the radiologic and clinical follow-ups were 17.7 and 107.9months, respectively. The male to female ratio was 1:1, with a median age at diagnosis of 9years. All AVMs were juvenile type, all perimedullary AVFs were high flow types (three type IVb and three type IVc), and one epidural AVF was associated with intradural venous drainage. Most cases (90%) were located in the cervical spine and conus medullaris. Acute neurological deterioration was identified in five patients; however, bleeding was identified in only one patient. Two cases were surgically treated, seven cases underwent embolization, and one case underwent radiosurgery. Three cases were completely obliterated, and their clinical states were improved (n=2; 66.7%) and stationary (n=1; 33.3%). Meanwhile, seven cases were incompletely obliterated, and their clinical states were improved (n=2; 28.6%), stationary (n=3; 42.8%), and aggravated (n=2; 28.6%). Pediatric spinal AVMs and AVFs were mostly complex and high flow types, and complete obliteration could not be satisfactorily achieved. Incompletely treated lesions should be closely followed up because they may worsen.