Endovascular Management of Type III Perimedullary Spinal Arteriovenous Malformations in Pediatric Population

<b><i>Background:</i></b> Spinal arteriovenous malformations (AVMs) are a cause of 20–30 of all spinal vascular malformation. The treatment option for the AVM depends upon the type of AVM. Here, we present a case series to discuss the type, management, and post-operative conclusion of the spinal AVMs. <b><i>Method:</i></b> Four patients with spinal AVMs were retrospectively reviewed. All 4 patients were with a nidus-type AVM. Treatment for all patients required embolization. Clinical features, imaging, treatment, and clinical results were observed. All 4 patient’s clinical outcome was assessed using the Modified Ranked Scale. <b><i>Result:</i></b> The follow-up after management showed that all four-patient recovered without any residual deficit. All four-patient scored zero (0) on the Modified Ranked Scale. <b><i>Conclusion:</i></b> Pediatric spinal AVMs are rare and require complex multimodal approach to achieve favorable outcomes.

Arteriovenous fistula of the filum terminale masqueraded as a failed back surgery syndrome – A case report and review of literature

Background: The filum terminale arteriovenous fistulas (FTAVFs) are a very rare type of spinal vascular malformation. Clinically, these lesions could present with a progressive ascending myelopathy also called FoixAlajouanine syndrome. Due to the rarity of these vascular malformation, some can be misdiagnosed, submitted to unnecessary spinal surgery, and even masqueraded as a failed back surgery syndrome. Based on the present case and related literature, we review all the cases with similar history and describe factors that should raise awareness for diagnosis of this spinal vascular malformation. Case Description: We present a case of a patient with a FTAVF at the level of L5-S1 that presented with a FoixAlajouanine syndrome. He had been previously submitted to a lumbar decompressive laminectomy without sustained improvement. After the identification and surgical treatment of the vascular malformation, he had progressive neurological improvement. Conclusion: FTAVF is a very rare spinal intradural spinal vascular malformation that can be masqueraded as a failed back surgery syndrome. In these cases, signs of ascending myelopathy should prompt awareness and vascular voids must be carefully evaluated in MRI.

The effect of adenosine in inducing cardiac arrest for endovascular treatment of paediatric high-flow brain and spinal vascular malformation

Introduction Endovascular treatment of high-flow vascular lesions with onyx may be very dangerous due to premature occlusion of the vein resulting from high flow-induced migration of this embolic substance without occlusion of the feeding vessels and the nidus. It is also dangerous because of the risk of occlusion of the normal vessels around the lesion. Inducing temporary cardiac arrest using adenosine may be helpful in limiting and minimizing these risks. However anaesthetic management of this procedure in children suffering from high-flow vascular lesions is difficult and challenging. Methods We report three paediatric patients with high-flow cerebrospinal vascular lesions that underwent endovascular treatment under general anaesthesia and temporary cardiac arrest with adenosine. Adenosine was administered in escalating doses to induce a few seconds of cardiac standstill and the onyx injection was synchronized with the onset of adenosine-induced cardiac standstill, the fistula being occluded by onyx. Results There were no complications in peri-procedural treatment in all three cases. Post-embolization angiography revealed complete obliteration of the lesion, and the patients’ neurological status progressively improved at follow-up. Conclusion Adenosine-induced temporary cardiac standstill was successfully used to facilitate safe and controlled endovascular onyx embolization of high-flow central nervous system vascular lesions.

The contributions of Otto Hebold and Julius Gaupp to the study of spinal vascular malformations: Original documents and historical context

Book chapters and journal articles dealing with spinal cord vascular malformations often reference Otto Hebold and Julius Gaupp, but frequently misrepresent the observations published by the two German authors in the late 19th century. The purpose of this paper is to provide a better appreciation of these important contributions based on abridged translations of original documents set in their historical context, notably regarding the landmark works of Brasch, Raymond and Cestan, and Lindenmann. It is concluded that Gaupp offered the first reliable description of a perimedullary arteriovenous fistula while the lesion reported by Hebold was not a spinal vascular malformation.

Presentation and outcomes of patients with thoracic and lumbosacral spinal epidural arteriovenous fistulas: a systematic review and meta-analysis

Background and purposeThoracolumbar and sacral spinal epidural arteriovenous fistulas (SEDAVFs) are an increasingly recognized form of spinal vascular malformation. The purpose of this study was to perform a systematic review of the demographics, clinical presentation and treatment results of thoracolumbar SEDAVFs.Materials and methodsPubmed, Scopus and Web of Science databases were searched from January 2000 to January 2018 for articles on treatment of SEDAVFs. Pooled data of individual patients were analyzed for demographic and clinical features of SEDAVFs as well as treatment outcomes.ResultsThere were 125 patients from 11 studies included. Mean age was 63.5 years. There was a male sex predilection (69.6%). Sensory symptoms including pain or numbness were the most frequently presenting symptoms. Fistula location was the lumbosacral spine in 79.2% and the thoracic spine in 20.8%. Involvement of intradural venous drainage was more common than extradural venous drainage only (89.6% vs 10.4%). Of the 123 treated patients, endovascular therapy was performed in 67.5% of patients, microsurgery in 23.6%, and combined treatment in 8.9%. The overall complete obliteration rate was 83.5% and did not differ between groups. Clinical symptoms improved in 70.7% of patients, were stable in 25%, and worsened in 1.7% with no difference between treatment modalities.ConclusionsThoracic and lumbosacral SEDAVFs often present with symptoms secondary to congestive myelopathy or compressive symptoms. Both endovascular and microsurgical treatments were associated with high obliteration rates and good clinical outcomes.

Cerebrovascular Disease

Part of the “What Do I Do Now?” series, Cerebrovascular Disease uses a case-based approach to cover common and important topics in the examination, investigation, and management of stroke, embolism, thrombosis, hemorrhage, and other critical presentations of cerebrovascular disease. Each chapter provides a discussion of the diagnosis, key points to remember, and selected references for further reading. For this edition, all cases and references have been updated and new cases have been added, including the following: ischemic stroke in cancer, posterior reversible encephalopathy syndrome (PRES), primary angiitis of the central nervous system, symptomatic spinal vascular malformation, and vascular dementia. Cerebrovascular Disease is an engaging collection of thought-provoking cases that clinicians can utilize when they encounter difficult patients. The volume is also a self-assessment tool that tests the reader’s ability to answer the question, “What do I do now?”

Thoracolumbar spinal vascular malformation as a rare cause of isolated intraventricular hemorrhage

Spinal vascular malformations are rare vascular lesions that most frequently present with back pain, radiculopathy, and/or myelopathy. Neurological decline is typically secondary to progressive radiculopathy, myelopathy, venous thrombosis, and stroke. Few case reports have described thoracolumbar spinal vascular malformations that present with both subarachnoid and intraventricular hemorrhage. This is the first reported case of a thoracolumbar spinal vascular malformation presenting with isolated intraventricular hemorrhage on initial imaging followed by acute and fatal rehemorrhage.