Single hole, high-flow, spinal cord peri-medullary arteriovenous fistula (PMAVF) in a child, treated with transarterial embolization: A case report.

Peri-medullary arteriovenous fistula (PMAVF) is a rare spinal vascular malformation that manifests as progressive neurologic deficits or hemorrhage in the spinal canal. We report a case of high-flow PMAVF in a child, with a single feeder artery and a large venous pouch, which was successfully treated with transarterial endovascular intervention. A 2-year-old boy was referred with a progressive 2-year history of myelopathy. The MRI revealed a large venous pouch at the midthoracic spinal cord with segmental surrounding edema. A spinal angiogram confirmed high-flow PMAVF with a single feeder artery from the anterior radiculomedullary artery, draining into the peri-medullary vein. The patient underwent transarterial embolization at the distal feeder artery, resulting in gradual motor strength improvement. PMAVF is classified as type IV spinal vascular malformation, usually presenting as a large, high-flow fistula with multiple feeders, although there was only one in this case. PMAVFs are intradural and may cause severe neurologic deficits due to mass effect, venous congestion, or hemorrhage, hence requiring prompt treatment. Treatment options for PMAVF include microsurgery, endovascular intervention, or a combination of the 2. Endovascular intervention with coil or liquid embolic material is considered first-line treatment for IVc PMAVF, and effective in type IVb with good clinical outcome. PMAVF is a rare spinal vascular malformation commonly manifesting as severe neurologic deficits but has the potential of favorable outcomes with endovascular therapy. This case demonstrates a unique angioarchitecture of high-flow PMAVF with a single feeder artery and large venous pouch, treated successfully with endovascular therapy.

An isolated Sinus Intracranial Dural Arteriovenous Fistula with Unusual Drainage Pattern.

Isolated sinus dural arteriovenous fistulas (DAVFs) involve a dural sinus with occlusion on both sides of the diseased sinus segment. Because of venous drainage refluxing from the isolated sinus into the cortical veins, all isolated sinus DAVFs are Borden type III or Cognard type Ⅲ/Ⅳ. Venous drainage typically involves temporo-occipital cortical veins or the superior petrosal sinus and tributaries of the petrosal vein. However, drainage veins involving the perimedullary venous system are extremely rare. Here, we present a case of Cognard type V isolated sinus DAVF successfully treated with balloon catheter and Onyx.

Autoimmune glial fibrillary acidic protein astrocytopathy complicated with low flow perimedullary arteriovenous fistula: a case report.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy and low-flow perimedullary arteriovenous fistulas (PMAVFs) may cause longitudinal widespread myelopathy. We report a middle-aged male patient with autoimmune GFAP astrocytopathy complicated with low flow PMAVFs disease, presenting with lower extremity weakness and dysuria. Magnetic resonance imaging (MRI) of the spinal cord revealed a significant longitudinal extent of T2 high signal from T11 to L1, with the lesion located proximal to the vascular territory supplied by the anterior spinal artery. Multiple patchy abnormal signals were seen adjacent to the anterior and posterior horns of the lateral ventricles bilaterally and at the centers of the semi-ovals on MRI of the cranial brain, with iso signal in T1Flair, the high signal in T2WI, and no high signal seen in Diffusion Weighted Imaging (DWI). Subsequently, the presence of anti-GFAP antibodies was detected in the cerebrospinal fluid (CSF), and the diagnosis of autoimmune GFAP astrocytopathy in conjunction with low-flow PMAVFs was confirmed through spinal digital subtraction angiography (DSA). This case report aims to increase neurologists' awareness of this disease and avoid missed or misdiagnosed cases that may lead to delayed treatment.

Peri-medullary anterior spinal artery aneurysm associated with cervical pial arterio-venous fistula.

Spinal arteriovenous fistulas represent a heterogenous group of pathologies and are divided into four categories. Type IV fistulas are further stratified into three groups (IVa, IVb and IVc) according to the number and dimensions of the fistulous vessels. Approximately 10% of these fistulas are associated with aneurysms. However, we are not aware of a previously reported case of an aneurysm associated with a Type IVa fistula with an anterior spinal artery (ASA) feeder at the cervico-medullary junction. We therefore describe our experience with a patient presenting with a ruptured aneurysm associated with a fistula in this location.

Case report: Delayed quadriplegia from traumatic carotid cavernous fistula: a rare case with perimedullary venous drainage.

Background: Carotid cavernous fistula (CCF) refers to the abnormal arteriovenous communication between the carotid system at the skull base and the sphenoid cavernous sinus, which is caused by trauma in almost 75% of cases. The drainage of venous blood to the spinal cord represents a distinctive mechanism, which is commonly observed in dural arteriovenous fistula (DAVF), and typically manifests clinically as progressive myelopathy. However, it is a rare occurrence in clinical practice that traumatic carotid cavernous fistula (TCCF) causes delayed quadriplegia through perimedullary venous drainage. Case presentation: We report the case of a 29-year-old male patient who was admitted to the hospital with a sudden onset of headache and quadriplegia. The patient had previously lost his right eye in a traffic accident 5 years ago. Cerebral angiography showed a high-flow direct CCF on the right side, accompanied by obvious drainage of cerebellar and perimedullary veins. We successfully performed coil embolization for the CCF, and the symptoms of the patient gradually improved after the operation. During follow-up at sixth-months, the patient regained the ability to walk independently. Conclusion: We experienced a rare case of TCCF with quadriplegia. Utilizing coil embolization, we achieved successful improvement in the patient's condition. However, the mechanism and the best treatment of CCF drainage through the perimedullary vein are still unclear. We need to further explore the pathophysiological information of CCF venous drainage.

Vascular Malformations of the Spinal Cord in Children.

Vascular malformation of the spinal cord in children is a rare and complicated disease spectrum. We will start from the basic spinal cord vascular anatomy and the controversial classification of this kind of disease. Then, we will elaborate the clinical manifestations, diagnostic imaging and treatment of pediatric spinal vascular malformations based on the practical experience of our center and from literature.

Cognard Type â ¤ Torcular Dural Arteriovenous Fistula: A Rare Entity.

Tentorial dural arteriovenous fistulas (DAVFs) are a dangerous subtype of fistula. Lawton et al classified this type of fistula into 6 categories based on specific operative strategies. Clinical manifestations of 6 types of tentorial DAVFs vary to some extent. Cognard type V DAVFs in the tentorial region are usually petrosal/superior petrosal sinus DAVFs. DAVFs with perimedullary venous drainage around the torcular herophili are rare. The veins of the posterior fossa are intricate and variable. There are anatomic communications between the perimedullary vein and cerebellar vein or veins of the brainstem. Almost all of the Cognard type V DAVFs are found in the posterior fossa. Here, we present a Cognard type V DAVF with a fistula located around the torcular herophili.

Spontaneous obliteration of a spinal perimedullary fistula.

We herewith report a young patient who had an incidental spinal vascular malformation of the cervicomedullary junction discovered during a work-up for anosmia. Angiography demonstrated a perimedullary spinal arteriovenous fistula with supply from lateral spinal arteries arising from bilateral V3 level segmental arteries. It was decided to manage the patient conservatively with magnetic resonance imaging monitored biannually. On a recent follow-up magnetic resonance, nearly 10 years later, we noted a subtle change in caliber and imaging characteristics at the posterior margin of the cervical medullary junction. Repeat digital-subtraction angiography showed no evidence of early venous filling from the previously involved branches. Microcatheter exploration of the right lateral spinal artery confirmed spontaneous occlusion of the spinal perimedullary arteriovenous fistula, without any persistent shunting. Spontaneous resolution of a spinal vascular malformation is rare; this case demonstrates the dynamic nature of shunting vascular malformations and that spontaneous obliteration of arteriovenous shunts is possible.

Spinal Dural Arterio-Venous Fistula: A Vital Differential Diagnosis to Consider for Myelopathy.

Introduction: Spinal dural arterio-venous fistula (SDAVF) is a rare and underdiagnosed cause of myelopathy which can result in a devastating neurological outcome if not properly managed. Case description: We report a case of SDAVF in a middle-aged man with gradual progressively deteriorating myelopathy and associated symptoms. This was first managed as demyelinating disease but was refractory to steroid therapy. Vigilant review of his spinal magnetic resonance imaging (MRI) scans showed dilated perimedullary veins, suspicious for SDAVF. The diagnosis was confirmed with catheter angiography. Neurological symptoms resolved after surgical treatment. Discussion: SDAVF can closely mimic demyelinating conditions such as transverse myelitis or multiple sclerosis. MRI finding of dilated perimedullary veins can be subtle and masked in the late stage, posing a diagnostic challenge for physicians. It is potentially curable after timely treatment. Conclusion: Clinicians should maintain a high level of suspicion for SDAVF and actively review all available radiological imaging for clues, particularly when there is a lack of response to treatment for other causes of myelopathy. LEARNING POINTS: Spinal dural arterio-venous fistula (SDAVF) can have clinical and radiological features similar to those of demyelinating disease, often causing a diagnostic dilemma for physicians. Neurological sequalae can be devastating if left untreated.Clinicians should be aware of this rare yet important differential diagnosis for myelopathy and its classic MRI findings (spinal cord oedema and dilated perimedullary veins).The gold standard for diagnosis is catheter spinal angiography. Treatment options include endovascular embolization and surgical ligation of the fistula.

Tentorial dural arteriovenous fistula with perimedullary venous drainage-associated cervical myelopathy: illustrative case.

BACKGROUND: Tentorial dural arteriovenous fistulas (DAVFs) with perimedullary venous drainage causing cervical myelopathy are very uncommon conditions with an extremely aggressive behavior. When the characteristic radiological clues are missing, the unspecific clinical picture may cause delay and make the diagnosis challenging. OBSERVATIONS: Here the authors report a case of a 58-year-old man who developed progressive spastic tetraparesis and dyspnea with an extensive mild enhancing cervical cord lesion initially oriented as a neurosyphilis-associated transverse myelitis. Acute worsening after steroid administration redirected the diagnosis, and a tentorial Cognard type V DAVF was elicited. The microsurgical disconnection process is described, and previously documented cases in the literature are reviewed. LESSONS: If a DAVF is highly suspected, it is important to consider the possibility of its intracranial origin, and spinal as well as cerebral arteriography must be performed.

Remote Intramedullary Hemorrhage in the Thoracic Spinal Cord Secondary to a Perimedullary Arteriovenous Fistula of the Distal End of Conus Medullaris Mimicking Filum Terminale Arteriovenous Fistula: A Case Report.

Perimedullary arteriovenous fistulas (PMAVFs) of the conus medullaris are rare and usually manifest with progressive myelopathy secondary to venous congestion resulting from retrograde arterialization of the draining vein into the spinal cord. We present a rare case of conus PMAVF presenting with remote intramedullary spinal cord hemorrhage in the thoracic cord. A 37-year-old woman was transferred to our institute due to sudden severe pain in the left lower leg and weakness of the lower extremities following progressive paresthesia of the lower extremities. Magnetic resonance imaging of the thoracic and lumbosacral spine revealed spinal cord congestion extending from the conus medullaris to the level of T6 with intramedullary hemorrhage at the level of T8-9 on the left side of the spinal cord. There were abnormal serpiginous intradural flow voids along the anterior surface of the spinal cord extending from the level of L2 to the lower cervical with venous varix at the level of T8-9, probably being the source of hemorrhage. Spinal angiography confirmed conus PMAVF at the distal end of the conus medullaris supplied by the sulco-commissural artery arising from the enlarged anterior spinal artery originating from the left T11 intercostal artery with cranial drainage through the dilated anterior spinal vein into the tortuous perimedullary veins up to the lower cervical level. The patient underwent successful endovascular treatment with N-butyl cyanoacrylate and had gradually improved until being ability to walk independently without residual pain of the left lower leg. We speculated that an increased venous flow into a varix may be considered an important risk factor of hemorrhage.

Spinal perimedullary arteriovenous fistula supplied by the artery of Desproges-Gotteron: A case report with literature review.

Spinal perimedullary arteriovenous fistula (PAVF) of the conus medullaris and cauda equina supplied by the artery of Desproges-Gotteron (ADG) is rare. The present study reports such a rare case and also presents a brief literature review. The patient described herein was a 31-year-old female. She suffered sudden weakness in the lower limbs, a condition which was then gradually aggravated. A physical examination revealed paraplegia, and urination and defecation difficulties. Spinal magnetic resonance imaging (MRI) and computed tomography angiography (CTA) revealed extensive perimedullary vein dilation with the involvement of the ADG as the main feeding artery. Digital subtraction angiography (DSA) confirmed a high-flow PAVF supplied by the ADG and artery of Adamkiewicz (AKA). Microsurgical treatment under intraoperative DSA assistance was performed. The PAVF was resected, and immediately following DSA confirmed the complete removal of the PAVF. Following discharge, the patient was prescribed rehabilitation exercises and gradually recovered. A follow-up CTA and MRI confirmed that the PAVF was cured, and her modified Rankin scale score was 2. Therefore, as demonstrated herein, microsurgery with intraoperative DSA assistance may be considered an effective treatment option for PAVFs with ADG.

Feasibility of Noninvasive Diagnosis of Spinal Vascular Diseases Using Time-Resolved Angiography With Stochastic Trajectories.

Background and Purpose: To determine the feasibility of time-resolved angiography with stochastic trajectories (TWIST) in the diagnosis of spinal dural arteriovenous fistula (SDAVF) and perimedullary arteriovenous fistula (PAVF). Methods: A total of 11 negative patients with TWIST examination were retrospective analyzed and then 18 patients with suspected spinal vascular diseases underwent TWIST. For negative patients, Adamkiewicz artery (AKA), great anterior radiculomedullary vein (GARV) and anterior spinal artery (ASA) were retrospective analyzed. In patients, the results of TWIST were compared with those of DSA. Results: The displaying rates of the ASA, AKA and GARV in 11 negative patients were 100, 90.9, and 90.9%, respectively. The AKA and GARV were separated on TWIST. Of 18 patients, 11 and three were diagnosed with SDAVF and PAVF, respectively. The spinal cord vascular malformation diagnosed on TWIST was consistent with DSA with an excellent intermodality agreement (Kappa = 0.92, p < 0.001). The feeding artery and side of all 11 SDAVF patients were displayed on TWIST and the results were consistent with DSA. For PAVF patients, the feeding artery in two patients and the sides as displayed on TWIST were consistent with DSA. Conclusions: TWIST enables the differentiation of the spinal artery and vein and the differential diagnosis of SDAVF and PAVF.

Remote Intramedullary Hemorrhage in the Thoracic Spinal Cord Secondary to a Perimedullary Arteriovenous Fistula of the Distal End of Conus Medullaris Mimicking Filum Terminale Arteriovenous Fistula.

Perimedullary arteriovenous fistulas (PMAVFs) of the conus medullaris are rare and usually manifest with progressive myelopathy secondary to venous congestion resulting from retrograde arterialization of the draining vein into the spinal cord. We present a rare case of conus PMAVF presenting with remote intramedullary spinal cord hemorrhage in the thoracic cord. A 37-year-old woman was transferred to our institute due to sudden severe pain in the left lower leg and weakness of the lower extremities following progressive paresthesia of the lower extremities. Magnetic resonance imaging of the thoracic and lumbosacral spine revealed spinal cord congestion extending from the conus medullaris to the level of T6 with intramedullary hemorrhage at the level of T8-9 on the left side of the spinal cord. There were abnormal serpiginous intradural flow voids along the anterior surface of the spinal cord extending from the level of L2 to the lower cervical with venous varix at the level of T8-9, probably being the source of hemorrhage. Spinal angiography confirmed conus PMAVF at the distal end of the conus medullaris supplied by the sulco-commissural artery arising from the enlarged anterior spinal artery originating from the left T11 intercostal artery with cranial drainage through the dilated anterior spinal vein into the tortuous perimedullary veins up to the lower cervical level. The patient underwent successful endovascular treatment with N-butyl cyanoacrylate and had gradually improved until being ability to walk independently without residual pain of the left lower leg. We speculated that an increased venous flow into a varix may be considered an important risk factor of hemorrhage.

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

BACKGROUND: 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. METHOD: 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. RESULT: 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. CONCLUSION: Pediatric spinal AVMs are rare and require complex multimodal approach to achieve favorable outcomes.

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.

BACKGROUND: 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. METHODS: 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. RESULTS: Compared with other methods, this method could identify the exact shunt point and was effective for certain shunt point obliterations. CONCLUSIONS: 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.

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.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Hemorragia subaracnoidea masiva durante la embolización de una malformación arterovenosa espinal de tipo iv. Complicación del tratamiento endovascular / Massive subarachnoid hemorrhage during type iv spinal arteriovenous malformation embolization. An endovascular procedure complication

Las malformaciones arteriovenosas espinales constituyen una entidad con una baja prevalencia y un diagnóstico en ocasiones complejo que precisa la colaboración de diferentes especialidades médicas. El abordaje multidisciplinar requiere una comunicación fluida entre servicios, ambiente laboral favorable y unas relaciones sociales correctas que traten de evitar la aparición de conflictos de intereses. Presentamos el caso de una mujer de 31 años remitida a nuestro departamento con una paraparesia asimétrica progresiva de 10 meses de evolución y disfunción vesical ocasional. Con el diagnóstico de malformación arteriovenosa espinal de tipo IV, se realizó una embolización programada tras la que se produjo una hemorragia subaracnoidea masiva debido a la laceración de la arteria espinal anterior que precisó una craniectomía bifrontal urgente. Discutimos el papel del neurocirujano en el tratamiento de las complicaciones derivadas del tratamiento endovascular de la patología espinal (AU)

Spinal arteriovenous malformations are rare diseases with a low prevalence and a complex diagnosis that usually requires the several neuroscience disciplines collaboration. Multidisciplinary approach requires fluid communication, favorable work environment and a correct social relationship, thus avoiding the conflict of interest appearance. We reported a 31 years old female referred to our department with progressive asymmetric spastic paraparesia for 10 months of evolution and occasional bladder dysfunction. With the diagnosis of a type IV arteriovenous malformation, a scheduled embolization was performed resulting in a massive subarachnoid hemorrhage due to the ASA laceration requiring an emergency bifrontal craniectomy. We discuss the neurosurgeońs role in complications during endovascular spinal vascular pathology treatment (AU)

Intradural lumbar radicular arteriovenous malformation mimicking perimedullary arteriovenous malformation of the conus medullaris: illustrative case.

BACKGROUND: Intradural radicular arteriovenous malformation (AVM) of the cauda equina is a rare entity of spinal AVMs. Because of the specific arterial supply of the conus medullaris and cauda equina, AVMs in this area sometimes present with confusing radiological features. OBSERVATIONS: The authors reported a rare case of intradural radicular AVM arising from the lumbar posterior root. The patient presented with urinary symptoms with multiple flow void around the conus medullaris, as shown on magnetic resonance imaging. Digital subtraction angiography demonstrated arteriovenous shunt at the left side of the conus medullaris fed by the anterior spinal artery via anastomotic channel to the posterior spinal artery and rich perimedullary drainers. There was another arteriovenous shunt at the L3 level from the left L4 radicular artery. Preoperative diagnosis was perimedullary AVM with radicular arteriovenous fistula. Direct surgery with indocyanine green angiography revealed that the actual arteriovenous shunt was located at the left L4 posterior root. The AVM was successfully treated by coagulation of feeding branches. LESSONS: Unilateral arteriovenous shunt fed by either posterior or anterior spinal artery at the conus medullaris may include AVM of the cauda equina despite abundant perimedullary venous drainage. Careful pre- and intraoperative diagnostic imaging is necessary for appropriate treatment.

Massive subarachnoid hemorrhage during type iv spinal arteriovenous malformation embolization. An endovascular procedure complication. / Hemorragia subaracnoidea masiva durante la embolización de una malformación arterovenosa espinal de tipo iv. Complicación del tratamiento endovascular.

Spinal arteriovenous malformations are rare diseases with a low prevalence and a complex diagnosis that usually requires the several neuroscience disciplines collaboration. Multidisciplinary approach requires fluid communication, favorable work environment and a correct social relationship, thus avoiding the conflict of interest appearance. We reported a 31 years old female referred to our department with progressive asymmetric spastic paraparesia for 10 months of evolution and occasional bladder dysfunction. With the diagnosis of a type IV arteriovenous malformation, a scheduled embolization was performed resulting in a massive subarachnoid hemorrhage due to the ASA laceration requiring an emergency bifrontal craniectomy. We discuss the neurosurgeons role in complications during endovascular spinal vascular pathology treatment.