Spinal Cord Arteriovenous Shunts Research Articles

Isolated spinal artery aneurysm: etiology, clinical characteristics, and outcomes.

Isolated spinal aneurysms (ISAs) are rare causes of subarachnoid hemorrhage (SAH), which encompass a highly heterogeneous group of clinical entities with multifarious pathogeneses, clinical characteristics, and treatment strategies. Therefore, knowledge about the ISAs remains inadequate. In this study, the authors present a comprehensive analysis of clinical data associated with ISAs at their institutions to enhance the understanding of this disease. Patients with ISAs confirmed by spinal angiography or surgery at the authors' institutions between 2015 and 2022 were included. Data regarding clinical presentation, lesion location, aneurysm morphology, comorbidities, treatment results, and clinical outcomes were reviewed. Seven patients with ISAs were included in the study. Among them, 4 patients (57.1%) experienced severe headache, and 3 patients (42.9%) reported sudden-onset back pain. Additionally, lower-extremity weakness and urinary retention were observed in 2 of these patients (28.6%). Four of the aneurysms exhibited fusiform morphology, whereas the remaining were saccular. All saccular aneurysms in this series were attributed to hemodynamic factors. Conservative treatment was administered to 3 patients, 2 of whom underwent follow-up digital subtraction angiography, which showed spontaneous occlusion of both aneurysms. Four patients ultimately underwent invasive treatments, including 2 who underwent microsurgery and 2 who received endovascular embolization. One patient died of recurrent SAH, while the remaining 6 patients had a favorable prognosis at the latest follow-up assessment. The morphology of aneurysms may be associated with their etiology. Saccular ISAs are usually caused by pressure due to abnormally increased blood flow, whereas fusiform lesions may be more likely to be secondary to vessel wall damage. The authors found that a saccular spinal aneurysm in young patients with a significant dilated parent artery may be a vestige of spinal cord arteriovenous shunts. ISAs can be managed by surgical, endovascular, or conservative procedures, and the clinical outcome is generally favorable. However, the heterogeneous nature of the disease necessitates personalized treatment decision-making based on specific clinical features of each patient.

Angiographic Index for the Treatment Efficacy and Functional Outcomes of Spinal Cord Arteriovenous Shunts: the Vertebral Blush Sign.

The functional outcomes in spinal cord arteriovenous shunts (SCAVSs) are usually unpredictable from current assessments of treatment results. We aimed to investigate and propose anew index marker, the vertebral blush (VB) sign, for assessing the treatment efficacy of SCAVSs. This retrospective cohort study enrolled patients diagnosed with SCAVSs between June 2012 and May 2021. The VB sign was defined as the angiographic finding of reappearance or enhanced contrast staining of the vertebral bodies fed by shunt-related arteries observed after shunt occlusion. The primary outcome was the improvement in motor/sensory disturbances or sphincter impairments within 1 year after treatment. The secondary outcome was shunt recanalization. VB sign characteristics and associations with outcomes were analyzed. Of 65patients with SCAVSs, 57were eligible for VB sign assessment; among these, there were 26with the VB sign and 31without the VB sign. Among vascular shunts perimedullary arteriovenous fistula showed the greatest difference in prevalence rate between those with and without the VB sign (33.3%, n = 9/27 versus 10.0%, n = 3/30; P = 0.031). On multivariable logistic regression analysis, SCAVSs with the VB sign had significantly more favorable outcomes than those without the VB sign (adjusted odds ratio, 5.61; 95% confidence interval, 1.48-21.23; P = 0.01). There was no relationship between the VB sign and secondary outcomes (P = 0.35). The VB sign is independently associated with functional recovery after shunt occlusion and could be an assessment tool for the treatment efficacy of SCAVSs.

High diagnostic performance of time-resolved MR angiography in spinal arteriovenous shunts

Background and purposeIn comparison with the limited efficacy of conventional MR imaging and the invasiveness of catheter-based digital subtraction angiography (DSA), time-resolved MR angiography (TR-MRA) has been proposed as a promising examination for early diagnosis of spinal arteriovenous shunts (SAVSs). This paper aims to investigate the diagnostic performance of TR-MRA with scan parameters optimized for SAVSs evaluation in a large number of patients. MethodsOne hundred patients with suspected SAVSs were enrolled. Each patient underwent preoperative TR-MRA with optimized scan parameters followed by DSA. The presence or absence of SAVS, the types and the angioarchitecture of SAVSs in the TR-MRA images were diagnostically analyzed. ResultsAmong the final 97 patients, 80 cases (82.5 %) were diagnosed and classified by TR-MRA as spinal cord arteriovenous shunts (SCAVSs; n = 22), spinal dural arteriovenous shunts (SDAVSs; n = 48), and spinal extradural arteriovenous shunts (SEDAVSs; n = 10). The agreement for classifying SAVSs between TR-MRA and DSA was excellent (κ = 0.91). The sensitivity, specificity, PPV, NPV, and accuracy of TR-MRA for the diagnosis of SAVSs were 100 % (95 % CI, 94.3–100.0 %), 76.5 % (95 % CI, 49.8–92.2 %), 95.2 % (95 % CI, 87.6–98.5 %), 100 % (95 % CI, 71.7–100.0 %), and 95.9 % (95 % CI, 89.9–98.4 %). The accuracy rates of TR-MRA for the detection of feeding arteries were 75.9 %, 91.7 %, and 80.0 % for SCAVSs, SDAVSs, and SEDAVSs respectively. ConclusionTime-resolved MR angiography showed excellent diagnostic performance for SAVSs screening. Additionally, this method can classify SAVSs and identify feeding arteries in SDAVSs with high diagnostic accuracy.

Arterioectatic Spinal Angiopathy of Childhood: Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology.

Pediatric patients with myelopathy expressing intradural spinal vascular ectasia without arteriovenous shunting were studied at four tertiary referral neuropediatric centers. Patients were identified by retrospective review of institutional records and excluded if spinal vascular pathology could be classified into a previously described category of spinal vascular malformation. Four patients meeting the study criteria were enrolled in the study. Clinical, magnetic resonance imaging, catheter-directed angiography, laboratory, histological and genetic data were analyzed to characterize the disease process and elucidate underlying pathomechanisms. Our study revealed a highly lethal, progressive multi-segmental myelopathy associated with a unique form of non-inflammatory spinal angiopathy featuring diffuse enlargement and tortuosity of spinal cord arteries, spinal cord hyperemia, and spinal cord edema (Arterioectatic Spinal Angiopathy of Childhood). The condition was shown to mimic venous congestive myelopathy associated with pediatric spinal cord arteriovenous shunts on MRI but to have distinct pathognomonic findings on catheter-directed angiography. Clinicopathological, genetic, and neuroimaging features, which are described in detail, closely overlap with those of mitochondrial disease.

The efficacy and deficiency of contemporary treatment for spinal cord arteriovenous shunts.

Contemporary treatments for spinal cord arteriovenous shunts are only based on clinicians' treatment experiences and expertise due to its rarity. We reviewed the clinical course of the largest multicentred cohort to evaluate the efficacy and deficiency of contemporary interventional treatments for spinal cord arteriovenous shunts. The clinical features, treatment results and clinical outcomes of 463 patients with spinal cord arteriovenous shunts were retrospectively assessed. The main outcome was the neurological deterioration that was evaluated based on the modified Aminoff and Logue scale. According to post-treatment digital subtraction angiography, complete obliteration was defined as disappearance of the intradural lesion, whereas partial obliteration was defined as any residual intradural lesion remaining visible and was further categorized as shunt-reduction obliteration (the nidus or shunt points were reduced) or palliative obliteration (only obliterated aneurysms or feeders). Cure rate was 40.6% for the whole cohort, 58.5% after microsurgery, and 26.4% after embolization. The curative resection was associated with non-metameric lesions, lesions with a maximum diameter <3 cm and lesions without anterior sulcal artery supply. The curative embolization was associated with fistula-type lesions, non-metameric lesions, and main drainage diameter <1.5 mm. The permanent treatment-related neurological deficits rate was 11.2% for the whole cohort, 16.1% after microsurgery, and 5.6% after embolization. The pretreatment clinical deterioration rate was 32.5%/year, which decreased to 9.3%/year after clinical interventions. Following partial treatment, the long-term acute and gradual deterioration rates were 5.3%/year and 3.6%/year, respectively. The acute deteriorations were associated with metameric lesions, craniocervical lesions, lesions with a maximum diameter ≥2 cm and residual aneurysm. Residual aneurysm was the only predictor of acute deterioration for non-metameric spinal cord arteriovenous shunts. The gradual deteriorations were associated with palliative obliteration, absence of pretreatment acute deterioration and intact main drainage. Although clinical risks of spinal cord arteriovenous shunts were reduced following clinical interventions, contemporary treatments for spinal cord arteriovenous shunt remains associated with considerable risks and incomplete efficacy. Individualized treatment plans should be adopted according to the angio-architectural features and major clinical risks of specific lesions. There is a higher opportunity for complete obliteration for lesions with simple angio-architecture. However, for most of spinal cord arteriovenous shunts with complex vascular anatomy, partial treatment is the only choice. For these patients, palliative obliteration targeting the aneurysms is recommended for reducing haemorrhagic risk, whereas shunt-reduction obliteration is necessary for non-haemorrhagic myelopathy. Contemporary treatment is ineffective in reducing haemorrhagic risk of incurable metameric spinal cord arteriovenous shunts.

Intradural spinal cord arteriovenous shunts in a personal series of 210 patients: novel classification with emphasis on anatomical disposition and angioarchitectonic distribution, related to spinal cord histogenetic units.

Few classifications of intradural spinal arteriovenous shunts (ID-SAVSs) have considered their anatomical localization in relation to their phenotype and angioarchitectonics. The authors propose another vision of ID-SAVSs allowing a reappraised classification based on analysis of the anatomical disposition, angioarchitecture, and histogenetic location of these vascular malformations. The radiological and clinical records of 210 patients with ID-SAVSs were retrospectively reviewed, considering their localization, vascular architectonics, and correlation with the 5 histogenetic units of the spinal cord. Among these, 183 files with complete data allowed precise analysis of the ID-SAVSs. Among these 183 files (162 and 21 cases with single and multiple lesions, respectively), different entities were identified: 13 pial macro arteriovenous fistulas (MAVFs), 92 pial micro arteriovenous fistulas (mAVFs), 33 superficial pial niduses, and 69 intramedullary niduses. Thirteen sulcal shunts (either fistulas or niduses) were considered subtypes of pial lesions. Among the 21 multiple cases, 11 were monomyelomeric while 10 were multimyelomeric. Pial lesions, either fistulas or niduses, were dominantly vascularized by pial arteries (anterior or posterior depending on the localization of the shunt) and occasionally (except for MAVFs) by transmedullary arteries. Pial niduses occasionally extended into the funiculus by recruiting intrinsic veins or by extension of the nidus itself inside the white matter. Intramedullary niduses were always vascularized by both centrifugal and centripetal feeders, respectively, from sulcal arteries (SAs) and pial arteries. Sulcal lesions are pial lesions located within the ventral median sulcus and vascularized by SAs and veins. Single or multiple ID-SAVSs can be part of various syndromes such as hereditary hemorrhagic telangiectasia, Parkes-Weber, RASA1, CLOVES, and spinal arteriovenous metameric syndromes. Histogenetic analyses revealed a specific distribution of each ID-SAVS in the 5 histogenetic units of the spinal cord: intramedullary niduses were found almost equally from cervical to thoracic units, while MAVFs and mAVFs were mostly found from thoracic to postcrural ones. Pial niduses showed intermediate features between intramedullary and fistulous lesions and were mostly distributed from brachial to crural segments. Precise analysis of the anatomical disposition of ID-SAVSs in relation to functional histogenetic units allows a better understanding of these lesions and improved therapeutic management.

Spinal cord arterio-venous shunts: From classification to therapeutic management.

Spinal Cord Arterio-Venous shunts (SCAVSs) are a rare disease. The aim of this paper is to describe how we classify and consider management of SCAVSs in relation to the location of the shunt focusing mainly on intradural SCAVSs. The anatomical features of the SCAVSs together with data provided by MRI and CT scans allow identification of four types of SCAVSs: paraspinal, epidural, dural and intradural ones. Clinical and neuroradiologic characteristics are described for each entity as well as the therapeutic endovascular management at our institution between 2002 and 2020. The therapeutic management of SCAVSs, and in particular of intradural shunts, remains mainly based on endovascular treatment as a first-choice approach. Understanding properly the lesional and regional vascular anatomy is mandatory to plan an appropriate therapeutic strategy and obtain good clinical results stable at long term follow up.

Treatment for Spinal Cord Arteriovenous Shunts: The Efficacy and Deficiency of Contemporary Clinical Interventions

Background: Contemporary treatments for spinal cord arteriovenous shunt (SCAVS) are only based on clinicians’ treatment experiences and expertise due to its rarity. We reviewed the clinical course of the largest multi-cantered SCAVS cohort for efficacy and deficiency of contemporary interventional treatments for SCAVSs. Methods: The clinical features, treatment results and clinical outcomes of 459 SCAVSs patients were retrospectively assessed. Partial obliteration of SCAVS was defined as shunt-reduction obliteration (the nidus or shunt points was reduced) or palliative obliteration (only obliterated aneurysms or feeders). Neurological deterioration occurred 2 weeks after treatment was defined as long-term deterioration and further classified as acute and gradual. Findings: Cure rate was 40·3% for whole cohort, 58·5% after microsurgery, and 25·6% after embolization. Non-metameric lesions, lesions with a maximum diameter < 3 cm and lesions without anterior sulcal artery supply were independent predictors for complete resection. Fistula-type lesions and main drainage diameter < 1·5 mm were independent predictors for complete embolization. Permanent treatment-related neurological deficits rate was 11·3% for the whole cohort, 16·1% after microsurgery, and 5·6% after embolization. The pre-treatment clinical deterioration rate was 32·4%/year, which decreased to 9·2%/year after clinical interventions. After partial treatment, the long-term acute and gradual deterioration rate were 5·3%/year and 3·6%/year, respectively. The acute deteriorations were associated with metameric lesions, craniocervical lesions, lesions with a maximum diameter ≥2 cm and residual aneurysms. The gradual deteriorations were associated with palliative obliteration, absence of pre-treatment acute deterioration and intact main drainage. Interpretation: Although clinical risks of SCAVSs were reduced after clinical interventions, contemporary treatments of SCAVS remains associated with considerable risks and incomplete efficacy. Individualized treatment plans should be adopted according to the angioarchitectural features and major clinical risks of specific lesions. Contemporary treatment is ineffective in reducing hemorrhagic risk of incurable spinal metameric arteriovenous shunts. Funding Statement: None. Declaration of Interests: None. Ethics Approval Statement: The study was reviewed and approved by the local ethics committee with waiver of informed consent from patients given its retrospective nature.

Natural history and management of spinal cord arteriovenous shunts in pregnancy: A monocentric series of 10 consecutive cases with emphasis on endovascular treatment

PurposeIntradural spinal cord arteriovenous shunts (IDSCAVS) are rare and constitute a challenging situation if symptoms occur during pregnancy. We present a series of ten such cases referred to our center: five cervical, four thoracic and one lumbar. MethodsWe retrospectively reviewed our global series of 215 IDSCAVSs between 2002 and March 2020 and found ten patients who had presented during pregnancy. Clinical, radiological and therapeutic data were studied. ResultsSeven shunts were AVM type niduses and three were micro AV-fistulae. All were associated with pial venous reflux and six hemorrhagic cases had pseudo aneurysms. Symptoms occurred mainly during the third trimester, 80% of patients presented with hemorrhage and spinal cord dysfunction. We embolized seven patients and proposed surgery in one, always after delivery: all recovered well. One woman declined treatment; one other was operated in emergency but did not improve. Mean follow-up was 3.9 years (0.5...19 years). ConclusionsDespite this small group of patients, our initial experience of IDSCAVSs diagnosed during pregnancy indicates that embolization is an effective management strategy if performed after delivery and a recovery period. Results indicate that IDSCAVSs seem to have a low risk of early rebleedings after the ictal event and may be closely followed up until delivery. The results obtained show good clinical outcome without long-term rebleeds. Women with known IDSCAVSs should not be discouraged from becoming pregnant, however it seems wise to embolize them before pregnancy in order to offer protection against risks during pregnancy.

Natural history of spinal cord arteriovenous shunts: an observational study.

The natural history of intradural spinal cord arteriovenous shunts is unknown. We performed an observational study in a consecutive patient cohort with symptomatic intradural spinal cord arteriovenous shunts who were admitted to three institutes to investigate the clinical course of this complex disease, which would provide valuable evidence to inform clinical decision-making. The clinical course of patients with symptomatic intradural spinal cord arteriovenous shunts from initial presentation to occurrence of clinical deterioration, initiation of treatment, or last follow-up was analysed. Patients with at least 1 month of observation were included in this study. Clinical onset and deterioration patterns were divided into acute and gradual. Annual and cumulative rates of clinical deterioration as well as their risk factors were analysed using Kaplan-Meier life table analysis and Cox proportional hazards model. To assess risks and benefits of treatment, post-treatment clinical courses were further assessed. Four hundred and sixty-six patients with a mean observational period of 36.9 ± 58.8 months were included; 56.7% of patients presented with acute onset, of whom 77.3% experienced spontaneous recovery. Age of onset older than 28 years, initial modified Aminoff and Logue scale of >3, mid-thoracic lesions and non-ventral lesions were independent predictors of failure for spontaneous recovery. The annual risk of general, acute and gradual clinical deterioration after onset was 30.7%, 9.9% and 17.7%, respectively. Risk of deterioration was highest in the early period after initial onset. Acute onset was the only independent risk factor [hazard ratio 1.957 (95% confidence interval, CI 1.324-2.894); P = 0.0008] of acute deterioration and gradual onset was the strongest predictor [hazard ratio 2.350 (95% CI 1.711-3.229); P < 0.0001] of the gradual deterioration among all the stratifying factors. After invasive treatment, complete obliteration was achieved in 37.9% of patients (138 of 364) and improved or stable clinical status was noted in 80.8% of patients. Forty-two patients (11.5%) experienced permanent complications. Overall post-treatment deterioration rate was 8.4%/year, and 5.3%/year if permanent complications were excluded. The natural history of symptomatic spinal cord arteriovenous shunts is poor, especially in the early period after onset, and early intervention is thus recommended. Initial onset pattern significantly affects the natural history of the lesion, which prompts a differentiated treatment strategy.

Intradural spinal cord arteriovenous shunts in the pediatric population: natural history, endovascular management, and follow-up.

Intradural spinal cord arteriovenous shunts represent a rare entity, particularly in the pediatric population, and clinical diagnosis can be challenging. We report the analysis of clinical, angioarchitectural, procedural, and follow-up data in a population of 36 children managed by our team between 2002 and 2017. Hemorrhage occurred in 26 children (72%). Age at onset was 9.22 ± 3.65 years. Lesions were located at the thoracic level in 16 cases, at the cervical level in 15 cases, and the thoraco-lumbar region in 5 cases. A genetic or metameric syndrome was associated in 18 children (50%). Glue embolization provided complete occlusion in 5 children, subtotal in 7, and extensive in 14 without intraprocedural complications. We observed clinical normalization in 11 children, improvement in 11 cases, and stability in 3. Four children worsened during the follow-up, and one child died. Endovascular staged glue embolization performed in experienced centers is safe in the treatment of pediatric intradural spinal cord arteriovenous shunts. Clinical and neuroradiological follow-up is mandatory, especially for pediatric patients.

Spinal cord arteriovenous shunts of the ventral (anterior) sulcus: anatomical, clinical, and therapeutic considerations.

Ventral sulcus spinal cord arteriovenous shunts (SCAVS) are rare vascular lesions that are located outside the spinal cord, are exclusively vascularized by the anterior spinal axis, and drain exclusively through the anterior spinal vein. We report the anatomical, clinical, and neuro-radiological features of SCAVS managed by our team. We conducted a retrospective study of patients with SCAVSs evaluated by the senior author of this report (GR) between 1981 and 2014. Data were collected by reviewing clinical notes and by a systematic analysis of spinal angiograms and MRI. Among 358 patients, we identified 8 patients (3 women) with ventral sulcus spinal cord arteriovenous shunts. Mean age was 30.5years. Six patients presented with progressive neurological symptoms, and two with acute neurological symptoms related to hematomyelia. Three shunts were located in the cervical cord, four in the thoracic cord, and one at the conus medullaris; there were two nidus type A-V shunts (AVMs) and six fistula type A-V shunts (AVFs). Seven patients were treated by endovascular therapy with glue embolization. Embolization led to anatomical cure in 5 cases, and a significant reduction of shunt volume and flow of more than 75% in 2 cases. In none of the cases we observed permanent morbidity. AVS of the ventral sulcus of the spinal cord are rare. Recognition of these lesions and precise localization of the anatomical space in which they are located, may allow a better understanding of their pathophysiology and clinical manifestations and guide proper therapeutic decisions.

Erratum

Journal of Magnetic Resonance ImagingVolume 41, Issue 2 p. 555-555 ErratumFree Access Erratum This article corrects the following: MRI and MRA of spinal cord arteriovenous shunts Stéphanie Condette-Auliac MD, Anne Boulin MD, Luca Roccatagliata MD, PhD, Oguzhan Coskun MD, Stéphanie Guieu MD, Pierre Guedin MD, Georges Rodesch MD, PhD, Volume 40Issue 6Journal of Magnetic Resonance Imaging pages: 1253-1266 First Published online: March 3, 2014 First published: 22 January 2015 https://doi.org/10.1002/jmri.24852AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat In 1, incorrect information was published on the opening page of the article. The information has been revised and the correct page appears below. We apologize for this error. Reference 1 Condette-Auliac S, Boulin A, Roccatagliata L, Coskun O, Guieu S, Guedin P, MD, and Rodesch G. MRI and MRA of Spinal Cord Arteriovenous Shunts. J Magn Imaging. 2014; 40: 1253– 1266. DOI 10.1002/jmri.24591. Wiley Online LibraryPubMedWeb of Science®Google Scholar Volume41, Issue2February 2015Pages 555-555 ReferencesRelatedInformation

MRI and MRA of spinal cord arteriovenous shunts

The purpose of this review is to describe the diagnostic criteria for spinal cord arteriovenous shunts (SCAVSs) when using magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA), and to discuss the extent to which the different MRI and MRA sequences and technical parameters provide the information that is required to diagnose these lesions properly. SCAVSs are divided into four groups according to location (paraspinal, epidural, dural, or intradural) and type (fistula or nidus); each type of lesion is described. SCAVSs are responsible for neurological symptoms due to spinal cord or nerve root involvement. MRI is usually the first examination performed when a spinal cord lesion is suspected. Recognition of the image characteristics of vascular lesions is mandatory if useful sequences are to be performed-especially MRA sequences. Because the treatment of SCAVSs relies mainly on endovascular therapies, MRI and MRA help with the planning of the angiographic procedure. We explain the choice of MRA sequences and parameters, the advantages and pitfalls to be aware of in order to obtain the best visualization, and the analysis of each lesion.

Spinal Cord Intradural Arteriovenous Fistulae: Anatomic, Clinical, and Therapeutic Considerations in a Series of 32 Consecutive Patients Seen between 1981 and 2000 with Emphasis on Endovascular Therapy

To review our series of intradural spinal cord arteriovenous fistulas (SCAVFs), analyzing symptoms and related angioarchitecture, and to study the morphological and clinical results of embolization. Thirty-two SCAVFs (in 22 adults and 10 children) were treated between 1981 and 2000. These lesions were classified as microarteriovenous fistulas (mAVFs) or macroarteriovenous fistulas (MAVFs) according to shunt morphology. Location, architecture, presenting symptoms, and age group were detailed. The selection of patients for endovascular versus surgical treatments was analyzed, as were the anatomic and clinical results obtained by embolization with n-butylcyanoacrylate. Clinical status was evaluated according to the Karnofsky Performance Scale score. Ten SCAVFs were found in the pediatric population (four mAVFs and six MAVFs). All four mAVFs presented with acute symptoms. Three mAVFs (two cervical and one thoracic) presented hematomyelia; in one patient with a thoracic AVF, subarachnoid hemorrhage was suspected. All six MAVFs were located in the thoracolumbar cord (five associated with hereditary hemorrhagic telangiectasias). Four of the six MAVFs presented with hemorrhage. In the adult population, there were 21 mAVFS (95%) and one MAVF (5%). Only two mAVFs were found in the cervical cord, all other shunts affecting the thoracolumbar region. Hemorrhage was present in 6 of the 22 cases seen in adults (27%). The symptoms of SCAVFs did not differ from those found in spinal cord arteriovenous shunts of nidus type. Pial venous reflux and congestion were the most frequently encountered features in both the adult and pediatric groups. Arterial aneurysms (different from false aneurysms) were not found in association with hemorrhagic presentation of SCAVFs. Mean follow-up in our series was 3.3 years. Of the MAVFs, 86% were embolized, with 67% cured. The others had more than 75% occlusion. All patients followed up improved significantly. Of the mAVFs, 48% were treated endovascularly. Successful embolization was performed in 75% of patients. One patient was not embolized because of vasospasm, whereas 67% percent of mAVFs were completely occluded, 22% were more than 90% occluded, and 11% were 75% occluded. Complementary surgery was deemed unnecessary. All patients with mAVFs improved significantly at follow-up. Transient complications occurred in 22% of all patients, with no permanent morbidity or mortality. No patient bled or rebled after embolization. Thirty-six percent of mAVFs were operated on because of anticipated technical difficulties for endovascular approach or distal localization of the shunt. Endovascular treatment of SCAVFs stabilizes, normalizes, or improves neurological symptoms in all patients at long-term follow-up, with no bleeds or rebleeds. Embolization of SCAVFs with glue is a safe treatment that compares favorably with other approaches and significantly improves the poor natural history of the disease.

Spinal cord arteriovenous shunts: from imaging to management

Spinal cord arteriovenous shunts: from imaging to management

Embolization of spinal cord arteriovenous shunts: morphological and clinical follow-up and results--review of 69 consecutive cases.

We sought to analyze the results of embolization in patients with intradural spinal cord arteriovenous shunts. The clinical and radiological files of 69 of a population of 155 patients treated with embolization between 1981 and 1999 were reviewed retrospectively. The patients' clinical status was evaluated according to Karnofsky Performance Scale score. Twenty-one (14%) of 155 patients were treated surgically because they were thought to be poor candidates for embolization. Twenty-four (15%) of 155 patients were considered untreatable with surgery or embolization; in these patients, follow-up was proposed, but only 8 of them were followed appropriately and remained stable after the first consultation. Forty-one (26%) of 155 patients consulted our group, but no follow-up could be obtained. In 69 (45%) of 155 patients, comprising 20 children and 49 adults, endovascular treatment was performed with the patients under general anesthesia and without provocative tests, mainly with acrylic glue, in 99% of these patients. The mean number of diagnostic and therapeutic sessions was 3.5 per patient, and the mean number of pure therapeutic sessions was 1.5 per patient. Follow-up ranged between 6 months and 18 years (mean, 5.6 yr). In 16% of patients, anatomic obliteration of spinal cord arteriovenous shunts was obtained. Embolization reduced more than 50% of the spinal cord arteriovenous shunts in 86% of cases. No recanalization was noted on follow-up angiograms. Good clinical outcomes were obtained in 83% of the patients: 15% of them were asymptomatic, 43% were improved, and 25% were stable. In 4% of patients, embolization failed to stabilize the disease. Transient deficits were seen after embolization in 14% of the patients, and permanent severe complications occurred in 4% of the patients (Karnofsky Performance Scale score <or=70). Mild worsening was seen in 9% of the patients (Karnofsky Performance Scale score, 80). No bleeding or rebleeding was seen after endovascular treatment was considered to have been completed. This study proves that embolization with acrylic glue is a therapeutic option that compares favorably with surgery or embolization with other agents (particles, coils, or balloons). It offers stable long-term clinical results, despite not necessarily achieving total cure. Studies of larger series with longer follow-up are necessary to confirm these encouraging therapeutic data.

Spinal cord arteriovenous shunts: from imaging to management

Spinal cord arteriovenous shunts (SCAVSs) are either fistulas or niduses that can be separated in four different groups according to their localization and relationship to the dura. Paraspinal AVSs are located outside the spine and are responsible for neurological symptoms because of cord compression by ertatic veins, venous congestion or arterial steal. Epidural shunts are located in the epidural space and drain in epidural veins with secondary intradural congestion. Dural shunts are embedded in the dura, produce a cord venous myelopathy after draining through veins that either pierce the dura far from a nerve root or accompany a nerve root. Intradural shunts affect the cord, the roots or the filum. Additionally, they can be classified according to their potential relationships with genetics, vascular biological features and angiogenesis into genetic hereditary lesions (hereditary hemorrhagic telangiectasia), genetic non-hereditary lesions (multiple lesions with metameric links) and single lesions (AVMs or micro AVFs). MRI and MRA are able to visualise SCAVS early after the onset of clinical symptoms. The type of shunt and its localization may remain difficult to be precise. Angiography remains the gold standard for analysis of the anatomical, morphological and architectural features necessary for therapeutic decisions in both paediatric and adult populations. In our series, embolisation is chosen in first intention whatever the type of shunt responsible for the clinical symptoms and glue is preferably used. In paraspinal, dural or epidural arteriovenous shunts, the goal of treatment should be complete closure of the shunt. A complete cure by embolization is rather easily achieved in paraspinal lesions. Failure of endovascular therapy in dural or epidural shunts must bring the patient to surgery. The prognosis of most intradural shunts seems better than previously thought, even after haemorrhage. In intradural spinal cord arteriovenous shunts, embolisation targeted towards the portions of the malformation felt to be responsible for the symptoms (venous congestion) or pointing to the point of rupture (false aneurysms) of the malformation, allows restoration of a new hemodynamic equilibrium between the malformation and the cord itself. Such targeted treatment offers long-term stabilisation or improvement to patients suffering from SCAVSs and good protection against (re) haemorrhages, with an acceptable morbidity. Cure of the shunt is not imperative to obtain these satisfactory outcomes. The clinical results obtained by such management compare favourably with those obtained by neurosurgery.

Classification of Spinal Cord Arteriovenous Shunts: Proposal for a Reappraisal???The Bic??tre Experience with 155 Consecutive Patients Treated between 1981 and 1999

Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances? We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions. All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease. It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.

Classification of Spinal Cord Arteriovenous Shunts: Proposal for a Reappraisal—The Bicêtre Experience with 155 Consecutive Patients Treated between 1981 and 1999

Abstract OBJECTIVE Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances? METHODS We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions. RESULTS All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease. CONCLUSION It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.