Dural Arteriovenous Malformation Research Articles

Dural AVM supplied by the ophthalmic artery

Dural arteriovenous malformations in the anterior cranial fossa are rare and are especially prone to haemorrhage. These lesions are usually treated by surgical excision. We report the embolization of an anterior cranial fossa DAVM using an endovascular approach via the ophthalmic artery.

A Novel Technique and New Grading Scale for the Embolization of Cerebral Vascular Malformations

Effective transarterial embolization of a dural arteriovenous fistula or pial arteriovenous malformation (AVM) requires penetration of a durable occlusive agent into the fistula or AVM nidus. Cyanoacrylate glue often cannot traverse the tortuous vessels that typically supply such malformations, leading to proximal occlusion and recruitment of collateral flow. Other embolic agents, such as polyvinyl alcohol particles, achieve better penetration, but their effects are short lived, often leading to recanalization. The authors sought to overcome these obstacles by developing a technique to enhance glue penetration into the fistula or AVM nidus itself. After placing a guide catheter in the proximal feeding artery, a microcatheter is advanced coaxially to its limit. As glue is injected through the microcatheter, a column of 5% dextrose in water (D5W) is pushed manually through the guide catheter lumen to propel the glue forward. This technique has been bench tested in a standard flow model of vascular malformations, using a pump capable of delivering various rates of D5W. It has also been validated in treating 17 patients with cerebral dural arteriovenous fistulae or AVMs, with real-time adjustment of D5W flow according to the extent of glue penetration. In the bench model, the extent of glue penetration, as graded by a new scale of liquid agent embolization proposed by the authors, correlated directly with the rate of D5W flow (P = 0.5, analysis of variance). In vivo, this technique has enhanced the penetration of glue into the fistula or AVM nidus, resulting in longstanding embolization of these malformations. Coaxial injection of D5W through the guide catheter can propel cyanoacrylate glue through tortuous feeding arteries and can enhance its penetration into dural fistulae and AVMs, leading to more effective endovascular treatment of these malformations.

Bilateral Petrous Ridge Dural Arteriovenous Malformations Treated by a Combination of Endovascular Embolization and Surgical Excision

We report the first case of bilateral complex petrosal arteriovenous malformations (AVM). The patient was treated by a combination of transarterial embolization and surgical excision on the right-sided AVM, and by transarterial and transvenous embolization on left-sided lesion.

Tentorial dural arteriovenous malformation manifesting as trigeminal neuralgia treated by stereotactic radiosurgery: a case report

Background Tentorial dAVMs as a cause of trigeminal neuralgia are extremely rare. Consensus exists that radical treatment of such high-flow dAVMs should be considered because of their high risk of bleeding. The authors present a case of a 50-year-old man who presented with symptoms of tic douloureux caused by tentorial dAVM, which was treated successfully by SRS. Case Description The patient consulted us with a 1-month history of harboring right trigeminal neuralgia. The cerebral angiography revealed a dAVM in the petrotentorial region and the MRI demonstrated a dilated petrosal venous varix compressing the root entry zone of the right trigeminal nerve. Gamma knife surgery was performed with a marginal dose of 18 Gy and a maximum dose of 30 Gy in a volume of 0.3 mL. The target point of the radiosurgery for this patient was a fistula identified by superselective angiography, enhanced computed tomography, and MRI. Follow-up MRI, 1 year after GKS, showed a thrombosed lesion, and the patient was able to end medication. There was no evidence of recurrence or adverse effects in the 3-year follow-up. Conclusion Stereotactic radiosurgery can play an effective role as a treatment modality for such unusual dAVMs.

Posterior cervical spinal cord infarction complicating the treatment of an intracranial dural arteriovenous fistula embolization

Acute ischemic infarction of the posterolateral bulbomedullary junction occurred in a 28-year-old man who underwent arterial embolization for the treatment of an intracranial dural arteriovenous malformation. The migration of the embolic agent in the posterior spinal artery via a peripheral anastomosis between the middle meningeal artery and the posterior meningeal artery was responsible for this complication. The cast of glue in the radiculopial system at the upper cervical level was visible on computed tomography scanning, and magnetic resonance imaging demonstrated circumscribed signal changes and restricted diffusion in the arterial territory of the bulbomedullary junction. The authors discuss the anatomical, clinical, and technical issues of this rare complication.

Intracranial dural arteriovenous fistula associated with progressive cervical myelopathy and normal venous drainage of the thoracolumbar cord: case report and review of the literature

Background Intracranial dural arteriovenous malformations draining into the perimedullary venous system are rare lesions. In these cases, the selective spinal catheterization of all vessels with potential of causing that malformation was negative, and additional cerebral angiography usually reveals the fistula. Because of venous congestion of the cord caused by the DAVF, a delayed drainage or stagnation of contrast material in the artery of Adamkiewicz was considered as a compelling angiographic disorder so far. Case Description We report about a 58-year-old patient with a DAVF of the right posterior fossa draining into the cervical and upper thoracic plexus of medullary veins, followed by progressive cervical myelopathy and a normal venous drainage of the artery of Adamkiewicz. Because of the failing endovascular treatment option, the neurosurgical intervention was performed. The fistula was explored and clipped without any complications. Immediately after operation, the patient reported an improvement of his neurological deficits. Conclusion About 38 cases of intracranial DAVFs draining into the perimedullary venous system are reported, but to our knowledge, this is the second one with a normal drainage of the artery of Adamkiewicz. The pathophysiological mechanisms, diagnostic procedures, and treatment modalities are discussed.

Embolisation par voie artérielle de malformations artérioveineuses durales intracrâniennes à l’aide d’ethylvinyl alcool copolymère (Onyx ®18)

To report our recent experience in transarterial embolisation of dural arteriovenous malformations (DAVM) using a new liquid embolic agent, Onyx18.6 patients were enrolled in this series. Clinical presentation was separated into 2 groups: aggressive (n=2), non aggressive (n=4). The DAVM was located at the superior sagittal sinus (n=1), at the transverse sinus (n=2), at the condylian canal (n=1), in the lesser sphenoid wing region (n=1), and in the tentorium region (n=1). The DAVM drained directly into a condylian or a cortical vein for three patients and into a venous sinus with cortical venous reflux for the three others. In this latter situation, the sinus was anatomically excluded from the normal brain venous drainage.A full brain angiogram including both internal carotid arteries, both external carotid arteries and ipsilateral vertebral artery, was performed before and after each treatment. The feeder chosen after a selective catheterisation for Onyx18 injection was always meningeal. Each treatment consisted of a single Onyx injection after one unique feeder catheterisation. Complete anatomical exclusion of the DAVM was achieved and demonstrated by the post treatment angiogram in all cases. There was no clinical complication after the treatment.Onyx18 used is a safe treatment for DAVMs. When its injection is performed in optimal conditions, it fills the total DAVM and its drainage vein or sinus after a single arterial feeder catheterisation.

Analysis of the best therapeutic alternative for intracranial dural arteriovenous malformations

The material for this research consisted of 93 patients with dural arteriovenous malformations (DAVMs) who were studied retrospectively with regards to therapeutic success and failure, who had undergone either neurosurgery, or embolization or a combination of both methods and whose disease was located in the cavernous sinus, the superior sagittal sinus, the transverse-sigmoid sinus of the anterior fossa and the tentorium. Thus, it was possible to arrive at the following conclusions: treatment of the DAVMs must be indicated, jointly, by an interventionist neuroradiologist and a neurosurgeon; DAVMs of the transverse-sigmoid sinus were better treated when a combination of both methods was used; DAVMs of the tentorium were also better treated with a combined method; the endovascular method ensured only a 50% chance of therapeutic success for DAVMs of the superior sagittal sinus; DAVMs of the cavernous sinus are better treated when the endovascular method was used with a transvenous approach, relative to the transarterial approach.

Paraplegia after Tethered Cord Surgery: An Uncommon Combined Anomaly of Spinal Arteriovenous Fistula and Sacral Lipoma— Case Report

The coexistence of spinal arteriovenous malformation (AVM) with congenital abnormalities is relatively common. However, the association of a spinal AVM and lipoma is rare. We present an adult patient with this combined anomaly and discuss the clinical relevance of this case. A 42-year-old Caucasian man with progressive paraparesis initially underwent surgery for a tethered spinal cord. Postoperatively, he became paraplegic. He improved gradually over an interval of 8 months and, at that point, worsened again. Subsequent angiographic study revealed a spinal dural arteriovenous fistula located at S1-S2. Additionally, an occipital dural AVM was discovered near the transverse sinus. The spinal arteriovenous fistula was excised along with the sacral lipoma. The occipital arteriovenous fistula was embolized successfully at a later time. The patient had immediate improvement in sensory symptoms after surgery. At a 9 month follow-up examination, he had regained the ability to walk with crutches, but his bladder dysfunction persisted. Recognition of co-existing vascular anomalies, such as spinal AVMs, is important in patients with tethered cords. The mechanisms involved in this patient's worsening neurological condition after release of the tethered cord are discussed.

Foix-Alajouanine Syndrome: An Uncommon Cause of Myelopathy From an Anatomic Variant Circulation

Foix-Alajouanine syndrome is a rare cause of myelopathy caused by dural arteriovenous malformation of the spinal cord, mostly lower thoracic and lumbar. Patients are usually over 50 years of age and can present with acute lower extremity dysesthesias or intermittent sciatica. Progression to paraplegia may be slow. Spinal angiography is needed for definitive diagnosis, based on the clues provided by the symptoms. If diagnosed early, vascular embolization or neurosurgical excision may be curative.

Acute subdural hematoma due to arteriovenous malformation primarily in dura mater: a case report

We report a 42-year-old female with alcohol addiction who suddenly died of subdural hematoma (SDH) caused by dural arteriovenous malformation (AVM). In autopsy, there was seen a massive SDH with a total weight of 181 g that covered an entire part of the left cerebral hemisphere, although either serious external injuries of the head or any visible internal injuries of the brain were observed. SDH subsequently resulted in the tonsillar, transtentorial and subfalcial herniations with a right-sided shift of the left-lateral and third ventricles, and the left thalamus as well. Histopathological examination on the serial sections cut from the falx cerebri revealed abnormal distribution of arteries and veins with various sizes, which were comprehensively highlighted by immunohistochemical stainings with α-SMA and CD31. Although a very point of bleeding was not identified even by careful histological observation, we concluded that dural AVM could be critical for acute SDH in the present case. The value of ethanol concentration examined in the samples from SDH supported that the lesion could be not chronic, but acute.

Complex tumors of the glomus jugulare: criteria, treatment, and outcome

Object Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors—multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome—that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. Methods In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. Conclusions Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intra-bulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.

Commentary: Complex tumors of the glomus jugulare: criteria, treatment, and outcome.

With rare exceptions, lesions of the glomus jugulare are benign, slow-growing paragangliomas. Advances in neuroimaging, skull base techniques, embolization, and anesthetic agents, along with emphasis on postoperative care have markedly improved the surgeons’ ability to remove these tumors and have improved outcome in an overwhelming number of patients who present with tumors of the glomus jugulare. Studies that include long-term follow up show the effectiveness of total removal in achieving a cure. There remains, however, a rare subgroup of glomus jugulare tumors that continue to present a formidable challenge to treatment. We have used the term “complex glomus jugulare tumors” to classify this group. These tumors are frequently considered to be extremely high risk or inoperable. One or more of the following criteria should be met to qualify a case for this designation: giant size, multiple paragangliomas (bilateral or ipsilateral), malignancy, catecholamine secretion, or association with other lesions such as dural arteriovenous malformations or an adrenal tumor. Previous treatment that resulted in adverse outcomes such as sacrifice of the carotid artery, necessity for radiation therapy, postoperative deficit, or adverse effect from embolization makes surgical intervention much more risky. We undertook the management of these tumors and report on the challenges involved in surgical removal, with commentary on various techniques and their outcomes. We emphasize the perioperative management of these lesions in special situations such as hypersecretion, multiple paragangliomas, and vascular involvement, and we discuss the rare malignant behavior of some of these tumors.

Cerebral MR venography

This paper illustrates the technique and clinical use of cerebral MR venography (MRV). Dural sinus thrombosis is detected by both two-dimensional time-of-flight (TOF) and three-dimensional phase-contrast (PC) techniques. Venous angiomas are demonstrated by the three-dimensional PC method. The arterial feeders, nidus and draining veins of cerebral arteriovenous malformation (AVM) can be visualized on contrast-enhanced TOF three-dimensional MR angiogram. The high-flow draining veins of cerebral and dural AVMs, vein of Galen malformation and carotid cavernous fistula are better seen on contrast-enhanced three-dimensional gradient–echo MRV.

Unruptured dural arteriovenous malformation of the transverse-sigmoid sinus presenting with focal symptoms and coma

We are presenting the case of a 63 year-old man with a dural arteriovenous malformation of the transverse sigmoid sinus who developed focal deficits followed by less localized symptoms such a disorientation, lethargy and eventually comatose status. Initial cerebral angiography showed retrograde filling of the cortical and deep cerebral venous system with marked delay in venous empting. Following embolization clinical symptoms completely cleared at the time that control angiography showed retrograde venous flow turning anterograde. Patient's symptoms recurred four months later when there was a relapse of retrograde cerebral venous drainage at the time he developed thrombosis of the superior longitudinal and right transverse sinuses. Sinus thrombosis and thrombosis of the central retinal artery were coincidental with hypercoagulability related to hyperhomocysteinemia. Since control angiography still showed persistence of the AV shunting radical excision of the involved dural sinuses was performed. The final outcome was excellent. The physiopathological mechanism responsible for neurological deficits in our patient most likely was ischemia of venous origin secondary to venous hypertension resulting from retrograde cerebral venous drainage. The clinical and angiographic presentation in few similar cases reported in the literature is reviewed.

Dural arteriovenous malformations of the skull base

Dural arteriovenous malformations (AVMs) are interesting lesions, which can present as diagnostic and therapeutic challenges. Such AVMs were initially described as located solely in the dural venous sinuses, but during the last decade researchers have discovered them in other locations such as the tentorium and foramen magnum. It is understandable that they can develop in other locations because numerous arteriovenous shunts normally occur all over the dura. Early reports emphasize that they are congenital; however, some investigators have proposed that they are acquired lesions arising after sinus thrombosis and trauma. They also have variable clinical features. From 1975 to 2000 we treated 400 AVMs, of which 46 were dural. Of these 46 dural AVMs, 18 could be classified as skull base AVMs. We restricted the designation of skull base AVMs to those AVMs located in the tentorium or adjacent to bones of the skull base. Two of these AVMs actually had their vascular nidus located in the bone. The common dural sinus AVMs were excluded from this category. The presenting clinical features of these skull base AVMs ranged from headache and pulsatile tinnitus to intracranial hemorrhage. They were treated by a combination of endovascular and microsurgical treatment. The majority of patients had a good clinical outcome. Skull base dural AVMs are an interesting clinical entity. Their clinical course, treatment and final outcome are variable depending on their location. They should be managed by a multidisciplinary approach.

Paravertebral Arteriovenous Malformation Supplied by Branches of the Iliac Arteries

Arteriovenous malformations of the spine and spinal cord can be classified into spinal cord arteriovenous malformations (AVMs) and fistulas (AVFs) and dural AVM and AVF occurring outside the dura but draining into the epidural veins called paravertebral AVM and AVF 1. Paravertebral malformations are rare arteriovenous communications outside the dura but draining into the epidural veins. These malformations produce symptoms from either venous congestion of the cord or cord compression from dilated epidural veins resulting in a myelopathy.We present a case of a patient with a lumbar paravertebral malformation treated successfully by endovascular occlusion of the feeders.

Chronic cerebral venous hypertension model in rats

Although cerebral venous hypertension is known as an important determinant factor for clinical manifestation and outcome in patients with dural arteriovenous malformation (AVM), the pathophysiology of that condition is not well understood. We have created a chronic rat model by cervical arteriovenous fistularization with jugular vein occlusion and examined effect of cerebral venous hypertension on cerebral blood flow regulation. This model may be suitable for investigating mechanisms of cerebrovascular alteration after venous hypertension.

Prenatal Diagnosis of Unusual Fetal Pial Arteriovenous Malformation

Cerebral arteriovenous malformations (CAVMs) are rarely diagnosed in utero. Most prenatal imaging of intracranial vascular malformations relates to Vein of Galen aneurysmal malformations (VGAMs) or Dural Arteriovenous Malformations (D-AVMs). We report a case of a fetal pial AVF with multiple fistulae and venous pouches, which appeared as an anechoic lesion on the prenatal ultrasound scan. The patient was asymptomatic with normal postnatal growth. No haemodynmaic disturbance was evident. Postnatal Computed tomography (CT), Magnetic Resonance Imaging (MRI) and catheter Digital Subtraction Angiography (DSA) confirmed the presence of a pial AVF. The angiographic findings and family history of nose bleeds suggests the diagnosis of Hereditary Hemorrhagic Telangiectasia. The largest AVF was embolized with tissue adhesive; the residual AVF subsequently removed by surgical excision.

Far posterior subtemporal approach to the dorsolateral brainstem and tentorial ring: technique and clinical experience.

Surgical access to the posterolateral mesencephalon or tentorial ring can be problematic, depending on the angle of the tentorium and associated venous structures. A far posterior subtemporal approach was developed that uses the wide opening of the tentorium and the option of supratentorial retraction of the cerebellum to provide an excellent angle of approach to this region. Details of this technique and a series of eight patients with lesions treated by this approach are presented. Seven cavernous malformations and one dural arteriovenous malformation were approached in eight patients. In this far posterior subtemporal approach, a horseshoe incision is centered slightly behind the ear, with the caudal extent of the craniotomy defined by the temporal fossa floor. The entry of the vein of Labbé into transverse sinus is identified intradurally. If the vein enters anteriorly in the exposure, it is mobilized. Retractors are then placed, and the tentorium is visualized and opened laterally, avoiding the trochlear nerve. A thin, tapered retractor can be used to retract the cerebellum posterolaterally away from the brainstem. This approach was used to treat eight patients, and their lesions were successfully resected. Seven patients experienced good outcomes, and one patient, who presented with Hunt-Hess Grade IV subarachnoid hemorrhage from a dural arteriovenous malformation, experienced a fair outcome. The far posterior subtemporal approach is effective for approaching carefully selected lesions of the posterolateral mesencephalon and tentorial ring.