Spinal Arteriovenous Malformations Research Articles

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.

Embolization for Perimedullary Arteriovenous Fistulae: Pioneering Experience in Peru

Background Perimedullary arteriovenous fistulae, or type IV spinal cord arteriovenous malformations, are very rare and not well-known lesions. This paper aims to present our endovascular experience with these lesions. Methods We report our experience with 4 patients with perimedullary arteriovenous fistulae, subtypes b and c (macrofistulae), exhibiting severe neurological impairment. The patients were treated with endovascular embolization. Results Complete fistula eradication was achieved in all of them. One complication occurred. We discuss the natural history, pathophysiology, clinical presentation, prognosis and embolization techniques, along with the angiographic and clinical outcomes. Conclusion Our experience with endovascular embolization as an upfront treatment allowed us to eradicate these lesions in a safe and effective way, arresting the clinical worsening and reversing partially or completely the neurological injury in most of our cases.

Spinal arteriovenous malformation in a pediatric patient with a history of congenital syphilis: a case report

BackgroundSpinal arteriovenous malformations in children are extremely rare and pose great risk for intraoperative hemorrhage. Congenital syphilis sometimes presents with vascular symptoms, however, there is little published on patients with a history of congenital syphilis presenting with spinal arteriovenous malformations.Case presentationA 15-month-old female with a history of congenital syphilis presented with urinary retention, fever, and subacute onset of paraplegia. MRI showed a lesion at T8-L1, angiogram was performed which confirmed the presence of a complex type IVc arteriovenous malformation and fistula from Artery of Adamkiewicz at L1-L2. It also showed peri medullary dilated veins and a pseudoaneurysm that compressed the spinal cord at T8-T10. Somatosensory evoked potentials and motor-evoked potentials were not recordable on the bilateral lower extremities prior to surgery. Once the patient was optimized for surgery, osteoplastic laminotomies from T6-T12 were performed. The dura was opened and the intradural, intramesenchymal hematoma was evacuated. There were two episodes of brisk arterial bleeding with hypotension during resection of the hematoma. The patient was taken to the angiography suite from the OR to successfully coil the large aneurysm. Intraoperative spinal cord monitoring remained undetectable in the bilateral lower extremities. The patient’s paraplegia remained unchanged from preoperative presentation.ConclusionCongenital syphilis may present with vascular changes that might impact surgical approaches and treatment outcomes in patients with spinal arteriovenous malformations. Preparation for massive transfusion and intraoperative monitoring are imperative in ensuring a safe perioperative experience.

Intraoperative Identification of the Shunt Point of Spinal Arteriovenous Malformations by a Selective Arterial Injection of Saline to Subtract Signals of Indocyanine Green: Technical Note

It is crucial to identify a shunt point for spinal arteriovenous malformation (AVM) treatment. For this purpose, some intraoperative supports have been reported-intravenous injection of indocyanine green (ICG), selective arterial injection of ICG, and selective arterial injection of saline with a high frame rate digital camera. However, there are difficulties in accurately identifying the shunt point, especially if the lesion has multiple feeders. The aim of this technical note was to report a novel method, selective arterial injection of saline to subtract signals of ICG, to precisely identify perimedullary arteriovenous fistula shunt points having multiple feeding arteries. After exposing the lesion, a 4-F catheter was cannulated into the origins of the segmental artery. ICG was injected intravenously as a first step, and then heparinized saline solution was flushed from the catheter. Compared with other methods, this method could identify the exact shunt point and was effective for certain shunt point obliterations. Despite having similar invasiveness, selective arterial injection of saline to subtract signals of ICG is superior to previously described techniques, such as selective arterial injection of ICG. Therefore, it will be useful in spinal arteriovenous malformation surgical treatment.

Surgical Treatment for Spinal Arteriovenous Malformation

In this paper, we outlined the diagnostic and treatment strategies for spinal arteriovenous malformations, focusing on dural and perimedullary arteriovenous fistula(AVF). In many patients with spinal arteriovenous malformations, the symptoms are non-specific. Therefore, we consider it is critical to detect the signal flow voids in the enlarged spinal veins using MRI. An accurate understanding of the vascular structures is indispensable for deciding appropriate treatment strategies. Hence, performing an angiography is essential. Regarding treatment, whether to select surgical or endovascular treatment for AVF depends largely on the institution's protocols. However, the treatment should always be based on an accurate diagnosis.

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.

Anesthetic considerations for cesarean delivery in a parturient with hereditary hemorrhagic telangiectasia: a case report

BackgroundHereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare autosomal dominant disorder characterized by mucocutaneous telangiectasia and arteriovenous malformations (AVMs). There are some anesthetic considerations for cesarean delivery in a parturient with HHT.Case presentationA 27-year-old parturient with pulmonary hemorrhage was admitted to our tertiary perinatal center. She was first diagnosed with HHT and a cesarean delivery using spinal anesthesia at 37 weeks of gestation was initially planned. However, magnetic resonance imaging (MRI) at 32 weeks of gestation revealed spinal AVM ranging from the thoracic to the lumbar levels. Thus, elective cesarean delivery under general anesthesia was planned. The parturient had an uneventful perioperative course.ConclusionsHHT should be considered as a differential diagnosis when parturients develop pulmonary hemorrhage. In a cesarean delivery of parturients with HHT, spinal MRI during pregnancy can help in deciding the anesthetic procedure to be used.

Spinal cord vascular diseases

Spinal vascular malformations include various entities, including spinal dural arteriovenous (AV) fistulas, acquired malformations and cavernous hemangiomas, and spinal arteriovenous malformations. AV fistula refers to aspinal cord vascular malformation in which there is a short-circuit connection between adural artery and aperimedullary vein superficial to the myelon. The condition is relatively rare, initial clinical symptoms are often misinterpreted, and adefinitive diagnosis is often delayed by 12months or more. However, early diagnosis is important to prevent progression of clinical symptoms, which may include paraplegia. Early magnetic resonance imaging (MRI) is helpful, showing intramedullary edema in the T2-weighted sequences and superficial, markedly dilated veins. Treatment consists of ligation of the AV fistula, either surgically or by embolization. Cavernous hemangiomas, consisting of alarge number of closely located immature blood vessels, are also relatively rare and have alow risk of bleeding (approximately 0.2-0.5%). Venous hemorrhage may also occur, resulting in clinical symptoms (including paraplegia).

Classifications of spinal arteriovenous malformations. Review

Our work is a review of various classification options for complex vascular pathology of the spinal cord, taking into account its historical changes as experience accumulates. Classifications of spinal arteriovenous malformations are very complex and the literature on this problem is presented in a small number of works. We conducted an analysis of 33 scientific articles in order to understand the classification of spinal arteriovenous malformations, its evolution and presented the most significant options for practicing neurosurgeons, endovascular surgeons. However, we did not find a classification that could satisfy the entire scientific community.

Bibliometric analysis of the top-100 most cited articles on the radiosurgical management of cerebral arteriovenous malformation

Background: Radiosurgery is an effective, alternative treatment modality in managing patients with cerebral arteriovenous malformations (AVMs). The present study aims to highlight the scholarly impact of the top-100 most cited articles on the radiosurgical management of AVMs.Methods: A title-specific search using the keyword “arteriovenous malformation” was conducted in the Scopus database. The outcome of the search was rearranged based on the citations count. Articles were categorized into four entities; clinical, gamma knife radiosurgery, linear accelerator (LINAC) radiosurgery, and proton beam radiosurgery. The exclusion criteria were applied to spinal or non-intracranial AVM, conference papers, non-English articles predominantly discussing the endovascular or microsurgical management.Results: The top-100 articles on the radiosurgical management of AVM were published between 1972 and 2016. Approximately one-third of the publications were produced between 1995 and 2000. The average citations per year for all papers were seven. The most-studied entity was pertinent to the clinical application of gamma knife radiosurgery in AVM (68%). The United States was the most active country in studying the radiosurgical application in AVM. The Journal of Neurosurgery published approximately one-third of the most-cited articles in the list. The top-3 most contributing authors, publishing 80% of articles in the list, were Lunsford et al.Conclusion: The radiosurgical management of AVMs evolved significantly throughout the years. Identifications of the publication trends facilitate the acquisition of evidence-based articles for authors investigating various radiosurgical techniques in the treatment of AVMs.

Combined surgical and endovascular approach to treat a ventrally located perimedullary arteriovenous fistula

Perimedullary arteriovenous fistulae (PMAVFs) (also called type IV spinal cord arteriovenous malformations) are rare lesions. They are located in the subarachnoid space or just under the pia. The shunt occurs between the anterior spinal artery (ASA) and/or posterior spinal artery (PSA) and a network of perimedullary veins. The aim of the treatment, surgical or endovascular, is to occlude the fistula. This article presents a unique treatment strategy of a demanding strictly ventral PMAVF in the lower thoracic cord. A posterior surgical approach with spinal cord rotation followed by direct puncture of a distally located arterial feeder was achieved. Precise identification of the fistula with superselective contrast injections was obtained and accurate catheterization of the venous pouch with a microcatheter was performed. Complete occlusion of the PMAVF was achieved with coils.

Morphological changes of a spinal arteriovenous malformation during pregnancy status

Morphological changes of a spinal arteriovenous malformation during pregnancy status

Microsurgical Resection of a Spinal Cord Pial Arteriovenous Fistula: 2-Dimensional Operative Video

We present a patient who was diagnosed 20 yr prior to current presentation with a spinal arteriovenous malformation. This patient had a 10-yr history of worsening back pain (and underwent lumbar fusion), urinary dysfunction leading to 3-yr dependence on intermittent catheterization, lower extremity paresthesias and pain, and progressive weakness with multiple falls, leading to walker then wheelchair dependence for mobility. Magnetic resonance studies showed extensive thoracic cord expansion and edema with enlarged spinal cord surface veins and flow voids extending from spinal levels T6 to the conus medullaris. Partial embolization at an outside institution elicited transient symptom improvement. Repeated spinal angiogram demonstrated persistent T10 pial arteriovenous fistula (AVF) supplied by the posterior spinal artery arising from the right T11 segmental artery as well as by the anterior spinal artery from the left T10 segmental artery. Because additional embolization carried significant risk, we planned open surgery with fistula resection. Informed consent for the surgery and video recording was obtained. The patient was placed in the prone position, and a radial artery access was obtained for intraoperative angiogram. Following a posterior T9-T11 laminectomy and dural opening, a pial dissection was performed to expose the AVF. Intraoperative indocyanine green angiography was used to assist in identifying the feeders and major drainage of the AVF. Post-AVF resection, a formal intraoperative radial access spinal angiogram demonstrated complete resection of the lesion with no residual shunt or early venous drainage. The patient improved significantly and, on last follow-up, is ambulating without any assistive devices.

Possibilities of endovascular embolization of spinal cord arteriovenous malformations using neurophysiological monitoring and provocative pharmacological tests

The study objective is to evaluate the results of pharmacologic provocative tests with neurophysiological intraoperative monitoring application during spinal cord arteriovenous malformation (AVM) embolization. Materials and methods . In the period from 2016 to 2018, 38patients with spinal cord AVM of different types (according the J. Anson and R. Spetzler classification) underwent endovascular surgery at the Federal Neurosurgical Center (Novosibirsk, Russia). Fifteen of these patients were operated using pharmacologic provocative tests with neurophysiological intraoperative monitoring of motor (MEP) and somatosensory evoked potentials (SSEP). Aminoff and Logue Disability Scale was used to estimate neurological deficit before and after surgery, additionally Medical Research Council Muscle Scale was used to estimate motor deficit. Results. In 15 patients, 37provocative samples with propofol and 38 samples with lidocaine with simultaneous registration of MEP and SSEP were performed. In the early postoperative period, a significant improvement in motor function was achieved in 2 patients, 3 showed a noticeable improvement, and 10 patients remained without dynamics. Urinary function improved in 2 patients, and in 13 remained unchanged. In 4 patients, the pharmacological test with propofol was positive, which was manifested by a decrease in the amplitudes of the MEP, and among the samples with lidocaine in 10 cases, a decrease in the amplitudes of the MEP was noted, with a simultaneous decrease in the amplitudes of the SSEP during 3 samples. In all patients, the decrease in the amplitudes of the evoked potentials was transient in nature and disappeared after a change in the place of administration of the pharmacological agent. This did not allow us to determine the sensitivity of the method for predicting persistent postoperative neurological deficit. To predict the development of motor deficiency in the early postoperative period, the specificity of lowering the amplitude of the MEP during the pharmacological test was: 57 % to reduce by 50 %; 87 % for a reduction of 80 % and 93 % for the complete disappearance of MEP. For the prognosis in the distant period (3 months after endovascular treatment), the specificity of decreasing the amplitude of the MEP by 50, 80 and 100 % was 46, 85 and 100 %, respectively. Conclusion. The use of neurophysiological monitoring and provocative tests is a safe method that allows you to make the optimal tactical decision in the endovascular treatment of spinal cord AVM. As a provocative test, it is recommended to use two pharmacological drugs (propofol and lidocaine). A 80 % decrease in MVP amplitudes or the disappearance of MVPs are more accurate criteria for the clinical outcome of spinal cord AVM embolization. The specificity of MVP in detecting an increase in motor deficiency in the early and long-term postoperative periods was maximum (93 and 100 %, respectively), if the disappearance of MVP was chosen as a criterion of pathology.

Spinal Arteriovenous Fistula, A Manifestation of Hereditary Hemorrhagic Telangiectasia: A Case Report.

IntroductionHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by arteriovenous malformations (AVM). HHT can have neurological manifestations.Case ReportA 32-year-old woman with a history of HHT presented to the emergency department with acute partial paralysis of the right leg, urinary retention, and right-sided back and hip pain. Magnetic resonance imaging of the spine demonstrated multiple, dilated blood vessels along the cervical spine, diffuse AVMs in the lumbar and thoracic spine, and a new arteriovenous fistula at the twelfth thoracic (T12) vertebral level. Her symptoms improved after endovascular embolization of the fistula.ConclusionSpinal AVMs are thought to be more prevalent in patients with HHT. Given the high morbidity of arteriovenous fistulas, early recognition and intervention are critical.

Four-dimensional digital subtraction angiography for exploration of spinal cord vascular malformations: preliminary experience

BackgroundThe precise understanding of the angioarchitecture of spinal vascular malformations (SVMs) is often difficult to reach with conventional digital subtraction angiography (DSA). The purpose of our study was to evaluate...

Use of an endoscope for spinal intradural pathology.

The endoscope has been increasingly used to treat various spinal diseases. However, the application of spinal endoscopy in an intradural lesion has been less common compared to that of an epidural counterpart. The authors reviewed existing literature describing spinal endoscopy as an aid in surgical treatment for intradural pathologies. Importantly, available literature indicated the safety and feasibility of spinal endoscopy for intradural lesions. Especially, an endoscope was utilized for intradural subarachnoid cystic lesions, tethered cord syndrome, extramedullary tumors, spinal arteriovenous malformations, and cordectomy for intractable pain. The results of this review should enhance further development and broaden application of an endoscope for various intradural pathologies.

Anterior chest pain as an unusual initial presentation in pediatric spinal arteriovenous malformations

Anterior chest pain as an unusual initial presentation in pediatric spinal arteriovenous malformations

Open surgery of complex long segment (giant) glomus spinal arteriovenous malformation

Spinal arteriovenous malformation occurring in the pediatric population (under 15 years) initially complain of pain as the first symptom which is often over looked. In almost all, diagnosis is delayed resulting in weakness. Angiogram provides correct diagnosis in all cases. Radical microsurgical excision tends to offer best long-term result.
 Here we present a 7-year-old girl referred to us for lumbar puncture with provisional suspicion of meningitis. She had history of fever for two days and signs of meningism were present. On examination, she was stable and had complains of headache. Her previous history suggested she had continuous backache since childhood and had visited multiple centers previously for the problem. A detailed work up revealed a long segment spinal space occupying lesion. CT angiogram showed spinal arteriovenous malformation. Laminoplasty and open microsurgical excision of arteriovenous malformation was performed. Her hospital stay was uneventful and was discharged in stable condition. Early recognition, accurate diagnosis and prompt surgical treatment may result in significant improvement of neurological function.

Clinical and angiographic results of long-term follow-up of patients after endovascular treatment for spinal arteriovenous malformations

Objective – to evaluate long-term results of treatment of patients with arteriovenous malformations AVM of the spinal cord. Materials and methods. A retrospective analysis of clinical and angiographic data of the results of treatment of patients with spinal AVM for the period from 2005 to 2019 was conducted. In SO «Scientific-Practical Center of Endovascular Neuroradiology NAMS of Ukraine» 47 patients were operated on endovascular method. The age of patients ranged from 11 to 62 years (middle age is 45.3 years). There were 29 men (61.7 %), 18 women (38.3 %). Liquid cyanoacrylate embolizant was used for endovascular intervention. The operations were performed with transfemoral access. Accoding the classification proposed by Anson and Spetzler in 1992 patients with type I were 31 (65.9 %), with ІІ type – 8 (17.0 %), with ІIІ type – 5 (10.6 %), with IV type – 3 (6.4 %). Magnetic resonance tomography and spinal angiography were used for diagnostics AVM.Results. Total exclusion of AVM from the bloodstream was achieved in 22 (46.8 %) cases. Out of the 31 (65.9 %) patients with type I AVM, 17 (54.8 %) had an early transient deepening of the neurological deficit. In this group, complete dissection of arteriovenous fistula was achieved in 16 (51.6 %) patients. In 8 (17.0 %) patients with type II AVM, complete exclusion of AVM was achieved in 5 (62.5 %) cases. In this case, 6 (75.0 %) patients were observed early postoperative deepening of neurological symptoms. In 5 (10.6 %) patients with type III AVM, total exclusion was achieved in 1 (20.0 %) case. Transient deepening of neurological deficit was noted in 3 (60.0 %) patients. In 3 (75.0 %) patients with type IV AVM, total embolization was achieved in 1 (33.0 %) observation. Neurological impairment occurred in 1 (33.0 %) patient. In all groups there were no fatalities.Conclusions. Angiography is the gold standard of diagnosis and dynamic monitoring of patients with spinal cord AVM. The use of endovascular treatment of AVM can stop the further progression of neurological deficits, which, however, is not always associated with the total exclusion of AVM. The best neurological improvement and total exclusion were achieved in patients with type I AVM. Radicality, efficacy and deepening of neurological deficiency depend on angioarchitectonics of AVM. The main condition for carrying out embolization is to achieve a safe level of catheterization of the afferent.