Spinal Arteriovenous Malformations Research Articles

A rare case of spinal involvement in hereditary hemorrhagic telangiectasia

IntroductionHere, we describe a rare case of a spinal arteriovenous fistula in a patient with known hereditary hemorrhagic telangiectasia (HHT) and spontaneous intraspinal hemorrhage. Furthermore, we provide a brief review of the literature on the formation of spinal arteriovenous malformations (AVM) in relation to this disease.Case presentationThe case involves a 54-year-old male with known HHT. At the age of 49, the patient experienced recurrent cystitis. Urological evaluation ruled the cause to be neurological and subsequent imaging revealed a thoracic AVM. Four years later, the patient was admitted to A&E with chest pain and loss of function of the lower extremities and right arm, suspicious for ruptured aortic dissection. Trauma-CT excluded this and a final diagnosis of ruptured spinal AVM was made. Seven months post-injury, a spinal angiography was performed confirming the AVM. The remaining AVM was embolized under general anesthesia with acceptable results.DiscussionSpinal involvement in HHT is exceedingly rare but remains an important differential diagnosis, especially when patients present autonomic symptoms as these could potentially progress to life-threatening complications. The literature and the presented case indicate the prudence of closing spinal AVMs in HHT in case of symptoms, including autonomic, such as bladder dysfunction.

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

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

Perioperative Management of Spinal Arteriovenous Malformation Embolization: Delayed Venous Thrombosis and Implications for Severe Back Pain.

The prognosis of untreated spinal arteriovenous malformations (SAVMs) is poor. Embolization plays an important role in the management of intramedullary SAVMs. Delayed aggravation due to spinal venous thrombosis following successful embolization has been reported; however, perioperative management strategies to prevent thrombosis have not been explored. We present our single-center experience of SAVM embolization and perioperative management, including anticoagulation. We retrospectively evaluated 18patients with SAVMs who underwent transarterial embolization. Perioperative anticoagulation therapy was administered to selected patients. We compared the characteristics of the patients, including perioperative management procedures, between those with and without postoperative worsening following embolization. Acute postoperative worsening within 1week occurred in 4 (22.2%) patients. Of these, immediate worsening was observed in one patient as aprocedure-related complication. Delayed worsening after 24 h was observed in 3 patients, caused by delayed venous thrombosis with severe back pain. Rescue anticoagulation for delayed worsening improved symptoms in two patients. Acomparison between patients with and without acute postoperative worsening revealed significant differences in age (median 46.5 vs. 26.5 years, p = 0.009) and the presence of postoperative back pain (75.0% vs. 0%, p = 0.005); however, there was no significant difference in use of selective anticoagulation (p = 0.274). The results of this study suggest that SAVM embolization can cause acute worsening due to postoperative venous thrombosis with severe back pain, which may be reversed by anticoagulation therapy. Back pain is an important finding that suggests venous thrombosis, and anticoagulation should be urgently administered.

Acute Subarachnoid Hemorrhage as a Result of Ruptured C1–C2 Cervical Spinal Arteriovenous Malformation: A Unique Case Report (P11-5.010)

Acute Subarachnoid Hemorrhage as a Result of Ruptured C1–C2 Cervical Spinal Arteriovenous Malformation: A Unique Case Report (P11-5.010)

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

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

Spinal arteriovenous malformation with a calcified nodule: illustrative case.

This article describes a rare case of cervical spinal arteriovenous malformation (AVM) mimicking a neurogenic spinal tumor. A 22-year-old female presenting with a C6-7 AVM with a calcification nodule experienced new-onset acute right upper radiculopathy associated with extradural compression of the spinal cord. Note that spinal AVMs with a calcified nodule are rare. Endovascular embolization is generally used to relieve the symptoms of AVM; however, this procedure cannot relieve cord compression, particularly in cases complicated by calcified nodules. This article discusses treatment options. Decompression surgery is preferable to endovascular embolization because it alleviates symptoms while preventing cord compression and minimizing the risk of recurrence.

Algorithm for Endovascular Treatment of Patients With Spinal Arteriovenous Malformations

Objective: To compare the endovascular treatment results in patients with spinal arteriovenous malformations (AVM) based on the proposed algorithm. Materials and methods: We retrospectively analyzed the endovascular treatment results in 72 patients with various types of spinal AVMs for 2014-2021. We formed 2 main groups of patients based on the developed indications for neurophysiological monitoring and provocative tests (NFM and PT): group 1 (n = 63) was treated according to the algorithm, and group 2 (n = 9) was treated before the algorithm was implemented. Group 1 was divided into subgroup 1.1 (n = 42) including patients with no indications for NFM and PT and subgroup 1.2 (n = 21) with patients indicated for NFM and PT. Subgroup 1.2 was further divided into subsubgroup 1.2A (n = 2) with patients indicated for NFM and PT yet to be informative due to severe neurological deficit and subsubgroup 1.2B (n = 19) with patients that had indications for and successfully underwent NFM and PT. We compared patients between groups 1 and 2, subsubgroup 1.2B and group 2 to evaluate the effectiveness of the algorithm (radical nature of the treatment, functional status assessment, complications). Results: Radical nature of spinal AVM treatment in group 1 was 79 % compared with 44 % in group 2 (P = 0.043). There was a significant improvement in motor function in group 1 compared with group 2 in each follow-up period (Р ≤ 0.007). Comparison of subsubgroup 1.2B and group 2 showed no significant differences (P = .05). The treatment led to complications in 5 patients (7 % of the total number of patients with spinal AVMs): 4 patients in group 2 and 1 patient in subsubgroup 1.2B. The effectiveness of the developed criteria was indirectly confirmed by difference in complications number between subsubgroup 1.2B and group 2 (P = 0.001). Conclusions: Group 1 showed better treatment results, significant clinical improvement, high radical nature of treatment, and a low percentage of complications compared with group 2. The proposed algorithm proved effective for main tasks of endovascular treatment of spinal AVMs.

BG09 RASA1 exon deletion in a neonate with vascular malformations

Abstract A full-term infant was noted to have a large, macular, pale-pink vascular birthmark across the left side of the chest, extending to the upper arm. Three smaller pale-pink irregular birthmarks on the elbow, thigh and flank were noted, as was an enlarged left upper arm. He had an oxygen dependency shortly after birth for which he required admission to the neonatal unit. A detailed history revealed that his father, paternal grandmother and aunt had similar birthmarks. His paternal aunt had an episode of bleeding from a spinal arteriovenous malformation (AVM) in her teens, which had been investigated with genetic testing by Sanger sequencing, but this did not identify a variant in RASA1. Following involvement from the dermatology, neonatology, genetics and radiology teams, investigations were organized. Findings included a high-flow vascular malformation with arteriovenous shunting on magnetic resonance imaging (MRI) of the left arm, normal MRI head and spine, and normal cardiac anatomy. Genetic testing by next-generation sequencing detected a heterozygous exon 21–23 deletion in RASA1. A diagnosis of capillary malformation–AVM syndrome (CM–AVM) was confirmed. Results of genetic testing of the father are awaited. Referral to a tertiary centre for consideration of management of AVM is underway. CM–AVM is a rare autosomal dominant condition. Pathogenic variants in RASA1 result in a lack of functional p120-RasGAP protein, which leads to abnormalities in angiogenesis, although this pathway is not well understood (Wooderchak-Donahue W, Johnson P, McDonald J et al. Expanding the clinical and molecular findings in RASA1 capillary malformation-arteriovenous malformation. Eur J Hum Genet 2018; 26:1521–36). Cutaneous vascular malformations, classically pink–red with a pale halo, are the most consistent cutaneous manifestation, but they may be subtle and can mimic other vascular birthmarks such as segmental infantile haemangioma, salmon patches or port wine stain. In our patient, pale halos were not apparent until 4 months of age. Identification is important as life-threatening complications from cutaneous vascular arteriovenous fistulas can occur (Wooderchak-Donahue et al.). This case highlights the importance of clarifying a family history, classifying vascular birthmarks and working with the multidisciplinary team. Genetic testing should be considered, even with previous negative analysis, as test sensitivity has improved and will now include analysis of EPHB4, which has also been associated with CM–AVM.

Syringomyelia secondary to thoracic spinal dural arteriovenous fistula presenting as paraparesis.

Spinal dural arteriovenous fistula (SDAVF) is also known as a type 1 spinal arteriovenous malformation, representing the most frequent vascular malformation of the spine. A high suspicion index is often required for the initial diagnosis of SDAVF because of subtle magnetic resonance imaging signs. We present the case of a patient with SDAVF associated with syringomyelia of the thoracic spinal cord and hypothesize that a fistula might induce intramedullary fluid accumulation due to venous hypertension, which leads to syrinx formation.

How I do it: surgery for spinal arteriovenous malformations.

Spinal arteriovenous malformations (AVM) are rare lesions. They may present with intramedullary hemorrhage or edema, often inducing severe neurological deficits. Active treatment of spinal AVMs is challenging even for experienced neurosurgeons. Anticipation of anatomy and AVM angiocharacteristics from preoperative imaging is key for successful treatment. Information gathered from MRI and DSA has to be then matched to intraoperative findings. This is a prerequisite for reasonably safe and structured lesion removal. We provide a structured approach for surgical treatment of spinal AVMs, supplemented by high-resolution video and imaging material.

Dissecting pseudoaneurysm in a spinal cord AVM treated by flow diversion: Technical note.

We report the case of a 29-year-old woman presenting with severe back pain and S1-radiculopathy. Digital subtraction angiography revealed a spinal cord arteriovenous malformation complicated by acute feeder dissection and pseudoaneurysm (PA) formation causing mass effect. After conservative management with limited angiographic and clinical improvement, selective treatment of the PA by flow diversion (FD) led to occlusion and shrinkage with the resolution of clinical symptoms. FD may be a feasible treatment option for spinal artery aneurysms in selected patients with favorable anatomy. The need for antiplatelet therapy is a limitation of its use with currently available devices.

429 Intramedullary Spinal Arteriovenous Malformations—Definition of Two Distinct Subtypes

INTRODUCTION: Intramedullary spinal arteriovenous malformations (SpiAVM) are complex lesions. Due to their rarity, reports of their management and outcomes are limited. METHODS: Our neurovascular database was reviewed SpiAVMs between January 1986 and December 2021. Demographic, clinical, radiographic, and outcome data were obtained. Eccentric SpiAVMs were defined as those with a significant portion presenting through the pia mater into the subarachnoid space, with large vessels coursing outside the parenchyma. Concentric SpiAVMs were defined as those embedded within the spinal cord parenchyma, not presenting to pia mater, and well circumscribed. RESULTS: Fifty-nine patients (mean [SD] age, 34 [15] years; female sex, 34) were identified. Patients exhibited myelopathy (88%), including pain (51%), paresis (88%), sensory deficits (46%), and bowel and bladder dysfunction (46%). Twenty-seven patients (46%) presented with hemorrhage. Forty-nine percent of lesions were located in the cervical spine and 51% in the thoracic spine. Thirty-five lesions (59%) were previously embolized. Twenty-eight patients (51%) were classified as eccentric and 27 (49%) as concentric SpiAVMs. Postoperative outcomes were similar with no significant differences (improved, 8/18 [44%] vs. 9/17 [53%]; unchanged, 8/18 [44%] vs. 7/17 [41%]; worse, 2/18 [11%] vs. 1/17 [6%]). Retreatment rates were not significantly different between both subtypes (eccentric, 9/28 [32%]; concentric, 6/27 [22%]). Eccentric and concentric SpiAVMs were associated with similar rates of complete resection (eccentric, 14/26 [54%], concentric, 18/26 [69%]). Of 55 classifiable lesions, 4 (7%) were determined to have a mixed phenotype, harboring characteristics of eccentric and concentric SpiAVMs. CONCLUSIONS: Intramedullary AVMs may cause significant symptoms. The eccentric and concentric subtypes have different angioarchitecture and outcomes. While complete resection is usually the preferred surgical objective, eccentric SpiAVMs are amenable to the pial resection that may achieve angiographic obliteration without complete resection.

CyberKnife Radiosurgery for Spinal Intramedullary Arteriovenous Malformations: A Single-Center Experience

Intramedullary spinal arteriovenous malformation (ISAVM, glomus type) is a type of spinal cord arteriovenous malformation, which is a rare disease known often to have a complex vascular supply interfering with that of the spinal cord, and is in complex anatomical relations with cord structures and nerve roots. Though microsurgical and endovascular treatment has mainly been the standard options, in high-risk cases with these treatments, stereotactic radiotherapy (SRT) might be the option of choice. We retrospectively reviewed 10 consecutive patients with ISAVM who received SRT using CyberKnife at the Japanese Red Cross Medical Center (Tokyo, Japan) from January 2011 to March2022. No case in this series suffered from hemorrhage after applying SRT. One case experienced neurological impairment 10years after SRT, which we attributed to venous congestion due to the remaining lesion. No case of radiation myelopathy was observed in this series. In one case, the nidus volume reduction and loss of flow voids were obvious, though improvement in the neurological outcome was not apparent. No radiological changes were observed in the other 9 patients. Even in lesions without radiological changes, no hemorrhagic events were observed for an average period of 4years. SRT may be a feasible option in treating ISAVM, especially for lesions in which microsurgical resection and endovascular treatment are inapplicable. To ascertain the safety and efficacy of this approach, further studies with more patients and longer follow-up is required.

A Classification Proposal for Spinal Vascular Lesions.

A Classification Proposal for Spinal Vascular Lesions.

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.

Unusual case of Parkes-Weber syndrome in a patient with spontaneous subarachnoid hemorrhage

Introduction. Parkes-Weber syndrome (PWS) is a complex and rare genetic disease of combined vascular malformations that primarily occur in the extremities and can involve the pelvic blood vessels. In extremely rare cases, the disease is manifested by endocranial and spinal involvement. The treatment of such patients represents a challenge for neurosurgical centers and requires a multidisciplinary approach. Case report. We present the case of a 46-year-old male patient admitted to the emergency department due to spontaneous subarachnoid hemorrhage (SAH), moderate flaccid paraparesis, and urinary incontinence. The patient was previously diagnosed with PWS, while the genetic evaluation proved the RASA1 gene mutation. Furthermore, he experienced a spontaneous SAH and was hospitalized 26 years ago, while six years ago, he underwent a right nephrectomy due to multiple hilar aneurysms of the right renal artery and its branches. Digital subtraction angiography of the endocranium was performed, which detected no aneurysmal dilatations or arteriovenous malformations (AVM). The magnetic resonance imaging recorded spinal intradural AVM in the vertebral levels between T12 and L3, which completely filled the dural sac. After the conservative treatment, there was a significant improvement in the patient?s neurological and clinical condition. Conclusion. To the best of our knowledge, this is the only case report of a patient with PWS who had a spinal intradural AVM and spontaneous SAH without high-output heart failure and with a history of a previous nephrectomy.

Thrombosis in hereditary hemorrhagic telangiectasia

Thrombosis in hereditary hemorrhagic telangiectasia

Differentiation between spinal dural arteriovenous fistulas, intramedullary spinal arteriovenous malformations, and perimedullary arteriovenous fistulas through magnetic resonance imaging

Differentiation between spinal dural arteriovenous fistulas, intramedullary spinal arteriovenous malformations, and perimedullary arteriovenous fistulas through magnetic resonance imaging

RETRACTED: Head-Mounted Augmented Reality in the Planning of Cerebrovascular Neurosurgical Procedures: A Single-Center Initial Experience

Augmented reality (AR) technology has played an increasing role in cerebrovascular neurosurgery over the last 2decades. Hence, we aim to evaluate the technical and educational value of head-mounted AR in cerebrovascular procedures. This is a single-center retrospective study of patients who underwent open surgery for cranial and spinal cerebrovascular lesions between April and August 2022. In all cases, the Medivis Surgical AR platform and HoloLens 2 were used for preoperative and intraoperative (preincision) planning. Surgical plan adjustment due to the use of head-mounted AR and subjective educational value of the tool were recorded. A total of 33 patients and 35 cerebrovascular neurosurgical procedures were analyzed. Procedures included 12 intracranial aneurysm clippings, 6 brain and 1 spinal arteriovenous malformation resections, 2 cranial dural arteriovenous fistula obliterations, 3 carotid endarterectomies, two extracranial-intracranial direct bypasses, two encephaloduroangiosynostosis for Moyamoya disease, 1 biopsy of the superficial temporal artery, 2 microvascular decompressions, 2 cavernoma resections, 1 combined intracranial aneurysm clipping and encephaloduroangiosynostosis for Moyamoya disease, and 1 percutaneous feeder catheterization for arteriovenous malformation embolization. Minor changes in the surgical plan were recorded in 16 of 35 procedures (45.7%). Subjective educational value was scored as "very helpful" for cranial, spinal arteriovenous malformations, and carotid endarterectomies; "helpful" for intracranial aneurysm, dural arteriovenous fistulas, direct bypass, encephaloduroangiosynostosis, and superficial temporal artery-biopsy; and "not helpful" for cavernoma resection and microvascular decompression. Head-mounted AR can be used in cerebrovascular neurosurgery as an adjunctive tool that might influence surgical strategy, enable 3-dimensional understanding of complex anatomy, and provide great educational value in selected cases.

Potential therapeutic targets and medications for arteriovenous malformations of the central nervous system

Arteriovenous malformations (AVMs) of the central nervous system are high-flow arteriovenous shunts that lead to considerable risks of hemorrhagic stroke and neurological deficits in young patients. Due to the complex angioarchitecture and their close anatomical relationship with the brain and spinal cord, the management of brain and spinal AVMs is challenging. Conventional invasive treatments, including microsurgery, endovascular embolization, and stereotactic radiosurgery, are associated with considerable risks and unsatisfactory efficacy. In addition, the lack of medications for AVMs represents an unmet clinical need. In recent years, the pathogenesis of AVMs has been progressively explored. The increased understanding of the mechanisms of the formation, progression, and rupture of AVMs has opened up several potential directions for AVM pharmacotherapy. In recent years, some promising drugs targeting angiogenesis, inflammation, vessel wall integrity, and the mitogen-activated protein kinase (MAPK)-extracellular receptor kinase (ERK) signaling pathway have been tested in a series of clinical investigations. In this review, we summarize the potential mechanisms, preliminary efficacy, and side effects of the candidate medications, including bevacizumab, minocycline or doxycycline, thalidomide, and trametinib, in the treatment of brain and spinal AVMs.